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Abstract:  Rhabdomyosarcoma of the perianal and perineal region is a rare sarcoma of childhood with a poor prognosis. The clinical findings are similar to those of condyloma accuminata, which makes it difficult to diagnose. There are a few reports of this disease in the dermatologic literature. We present a 15-month-old girl with a polypoid anal mass diagnosed as embryonal rhabdomyosarcoma and a review of the current literature.