A case of a 65-year-old caucasian woman with progressive hemifacial atrophy (Parry-Romberg syndrome) is reported. The atrophy had begun ten years before. At time of presentation, she was suffering from facial pain and diplopia. She presented with severe enophthalmos associated with eyelid atrophy, loss of cilia and mild blepharoptosis. There was a patch of alopecia and atropic areas of skin, recognised as scleroderma "en coup de sabre". Ophthalmic examination showed miosis. Horner's syndrome and ocular motility disturbance. Biomicroscopic examination of anterior and posterior segments was normal; there was no heterochromia and no disc atrophy. Aesthetic surgical treatment was proposed. The etiology of hemifacial atrophy remains controversial, and its relations with scleroderma and autonomic nervous system disorders are discussed.