A case report of congenital embryonal rhabdomyosarcoma of the superior extremity (right shoulder) is described. This patient was the first and only child of a 24 year-old woman who had been previously treated for sterility. The diagnosis was done by echography during the 36 th week of gestation. This full term white boy was born by cesarean section in reason of tumor size. First he was treated surgically. Then, a cyclic chemotherapy (VAC) was performed. After 4 courses, local recurrence led to a second surgical excision followed by a new cyclic chemotherapy (IVA). The treatment had an acceptable tolerance. Seven months later the child is free disease alive.