The authors describe a case of progressive facial hemiatrophy in a woman aged 26 years, coming from the Isfarin region of Tadzhikistan. The patient views herself as being ill for 14 years, since the moment of an epileptic attack with tonic and clonic convulsions. Approximately at the same time she noted a small dry ulcer on the left on the vertex. The ulcer slowly increased, followed by skin atrophy. The disease progressed for 4 to 5 years. At present to the left there are folds in the form of scars on the face. The skin is thinned, united with the bones in the frontal and parietal areas, the subcutaneous fat is atrophic. The lips and nose at the left are subatrophic. Negligible enophthalmos, hemiatrophy of the tongue at the left. Alopecia. A certain deterioration of memory and reduction of the critical attitude are recorded. The patient is in a state of euphoria. Left-sided anosmia. The left auricular floor is subatrophic, hearing is almost lacking. Diffuse elevation of the tendinous reflexes of the limbs on the left side. X-ray signs of osteoporosis of the bones of the cranial vault on the left.