Background: Progressive facial hemiatrophy is characterized by a slowly progressive atrophy affecting one side of the face. Its clinical manifestations are mainly neurological and ocular. The most common neurologic complications include migraine, trigeminal neuralgia and epilepsia.
Case Report: A 27-year-old male presented with a 6-year history of progressive facial hemiatrophy involving the right side of face, associated with mastigatory spasm and trigeminal neuralgia. Computed tomography and magnetic resonance imaging of the brain and electrophysiological investigations were normal. Histopathological examination of a skin biopsy specimen from the chin revealed showed perivascular and interstitial lenfosit infi ltration and focal perifolliculit.
Conclusions: We defi ned the craniofacial fi ndings and neurological symptoms in our 27 year old patient that had no previous diagnosis with slowly progressive disease.

Keywords: Romberg’s disease, Trigeminal Neuralgia, hemimasticatory spasm