Rapp-Hodgkin syndrome is an uncommon, autosomal dominant condition characterized by distinctive craniofacial anomalies, cleft lip or palate, poor dentition, poor hair growth, dystrophic nails, and hypohidrosis, and hypospadias in boys. Since the original report in 1968, fewer than 20 other patients have been described. We report a new sporadic case, a 12-year-old boy who had erythrodermia and scaling skin at birth, and later developed most of the symptoms and findings previously described.