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Abstract: Rapp-Hodgkin syndrome is an uncommon, autosomai 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 originai report in 1966, fewer than 20 other patients have been described. We report a new sporadic case, a 12-year-oid boy who had erythrodermia and scaling skin at birth, and later developed most of the symptoms and findings previously described.