Introduction: The Parry-Romberg syndrome is a clinical condition in which there is progressive facial hemiatrophy which may be associated with neurological, ocular, cutaneous and auto-immune disorders. It is of unknown aetiology, although many theories have been put forward.
Clinical case: We present a case of left facial hemiatrophy associated with ipsilateral lingual hemiatrophy and signs of cortico-cerebellar dysfunction, the neuroimaging findings (NMR) and clinico-radiological follow-up covered a period of over 10 years.
Conclusion: The data on the clinical course and neuroimaging, together with our review of the literature, suggest that the Parry-Romberg syndrome may be considered to be a dysgenetic process which may originate during the first stages of CNS embryogenesis.