Parry Romberg syndrome is an idiopathic disease, characterized by subcutaneous tissue atrophy. Its main clinical sign is progressive facial hemiatrophy including: skin, subcutaneous tissue, nerves, muscle and/or bone. Several theories have been mentioned, among them the possible hereditary and genetic origin of this disorder, as the manifestation of this disease sometimes is seen in members of the same family. We present a case: a patient with late presentation Parry Romberg, with right side face atrophy associated with refractory epilepsy and family history of a brother who presents right side face atrophy as well and a marked scleroderma in coup of saber.