Abstract
Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that include skin, ocular, and neurological disorders. This study describes the case of a 16-year-old girl that came to observation for the treatment of a congenital alopecia causing great psychological distress. After two expansion procedures the hairless patch was restored with high patient satisfaction. The case met all the criteria for definite diagnosis of ECCL.