We describe a term baby girl with (CHARGE syndrome coloboma, heart anomaly, choanal atresia, retardation, and genital and ear anomalies association) who had persistent complete absence of the Moro reflex but preservation of other primary neonatal reflexes. A computed tomography scan revealed bilateral absence of the semicircular canals and hypoplastic vestibules. We suggest that isolated absence of the Moro reflex may reflect underlying abnormalities of the vestibular system and may, therefore, not necessarily be associated with a generalized disturbance of the central nervous system as is usually the case. This suggestion has implications for the diagnostic and prognostic relevance of an isolated absent Moro reflex.