2027 ICD-10-CM Diagnosis Code Q75.3Macrocephaly
ICD-10-CM Codes›Q00-Q99›Q65-Q79›Q75
- Billable — Valid for Submission
- POA Exempt
- Not Chronic
Q75.3 is a billable ICD-10-CM diagnosis code for macrocephaly. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 564 through 566. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Musculoskeletal congenital conditions.
Code Identity
Code Classification
Present on Admission (POA)Billing
Q75.3 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Congenital malformations and deformations of the musculoskeletal system (Q65-Q79).
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Agenesis of corpus callosum, macrocephaly, hypertelorism syndrome
- Atkin Flaitz syndrome
- Autosomal dominant intellectual disability, craniofacial dysmorphism, macrocephaly, hypotonia syndrome due to H1-4 mutation
- Clark Baraitser syndrome
- Colobomatous microphthalmia
- COMMAD syndrome
- Complete deafness
- Congenital abnormal shape of rib
- Congenital deafness
- Congenital dysplasia of nail unit
- Congenital macrocephaly
- Congenital pectus excavatum
- Cutis laxa, autosomal recessive
- Dandy-Walker syndrome
- Disorder of ornithine metabolism
- Facial dysmorphism, macrocephaly, myopia, Dandy-Walker malformation syndrome
- Fryns macrocephaly
- Genetic syndromic childhood obesity
- Global developmental delay, alopecia, macrocephaly, facial dysmorphism, structural brain anomalies syndrome
- Intellectual disability, coarse face, macrocephaly, cerebellar hypotrophy syndrome
- Intellectual disability, macrocephaly, hypotonia, behavioral abnormalities syndrome
- Intellectual disability, seizures, macrocephaly, obesity syndrome
- Left ventricular myocardial noncompaction cardiomyopathy
- Macrocephaly
- Macrocephaly and developmental delay syndrome
- Macrocephaly, intellectual disability, autism syndrome
- Macrocephaly, intellectual disability, left ventricular non compaction syndrome
- Macrocephaly, intellectual disability, neurodevelopmental disorder, small thorax syndrome
- Macrocephaly, short stature, paraplegia syndrome
- Macroencephaly
- MACS syndrome
- Megalencephaly capillary malformation
- MOMO syndrome
- Overgrowth, macrocephaly, facial dysmorphism syndrome
- Pectus deformity of chest
- Pectus excavatum
- Pectus excavatum, macrocephaly, dysplastic nails syndrome
- Progressive macrocephaly
- Relative macrocephaly
- Seizures, scoliosis, macrocephaly syndrome
- Ventricular myocardial noncompaction cardiomyopathy
- X-linked intellectual disability, macrocephaly, macroorchidism syndrome
- X-linked recessive intellectual disability and macrocephaly with ciliary dysfunction syndrome
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to Q75.3: its own notes plus those printed at Q75 and Chapter 17. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- congenital malformation of face NOS (Q18.-)
- congenital malformation syndromes classified to Q87.-
- dentofacial anomalies [including malocclusion] (M26.-)
- musculoskeletal deformities of head and face (Q67.0-Q67.4)
- skull defects associated with congenital anomalies of brain such as:
- anencephaly (Q00.0)
- encephalocele (Q01.-)
- hydrocephalus (Q03.-)
- microcephaly (Q02)
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
Notes
General instructions on how these codes are used.
- Codes from this chapter are not for use on maternal records
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name Q75.3, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming Q75.3: they are not reported together with it, unless the two conditions are unrelated.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Megalencephaly
a congenital abnormality in which the occipitofrontal circumference is greater than two standard deviations above the mean for a given age. it is associated with hydrocephalus; subdural effusion; arachnoid cysts; or is part of a genetic condition (e.g., alexander disease; sotos syndrome).
Patient EducationClinical
Craniofacial Abnormalities
Craniofacial is a medical term that relates to the bones of the skull and face. Craniofacial abnormalities are birth defects of the face or head. Some, like cleft lip and palate, are among the most common of all birth defects. Others are very rare. Most of them affect how a person's face or head looks.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert Q75.3 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About Q75.3Overview
What is the ICD-10 code for macrocephaly?
The ICD-10-CM code for macrocephaly is Q75.3 (sometimes written as Q753). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is Q75.3 (Other congenital malformations of skull and face bones) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report macrocephaly on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does Q75.3 group to?
When macrocephaly is the principal diagnosis on an inpatient stay, it groups to MS-DRG 564, 566, 565, with relative weights from 0.7374 to 1.4909 depending on complications. Higher weights mean higher Medicare reimbursement.
Is Q75.3 exempt from POA reporting?
Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for macrocephaly on inpatient claims. The code appears in the Congenital malformations and deformations of the musculoskeletal system (Q65-Q79) range of the CMS exempt list.
Can Q75.3 be reported with a code from Q18?
Not as a rule. The Excludes1 note at Q75, which applies to Q75.3, lists "congenital malformation of face NOS (Q18.-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
