Proteus syndrome associated with a sacrococcygeal teratoma; a rare combination

We report on an 5-year-old boy with a Proteus syndrome known since his second year of life. Hidden in a lipomatous tumor we excised a sacrococcygeal teratoma which must have been present since birth. This is the first case, out of about 50 published Proteus cases, of a patient with Proteus syndrome and sacrococcygeal teratoma.