A unique case of congenital embryonal rhabdomyosarcoma of the hand in a patient with Apert's syndrome is presented. Immediately after birth a 1 cm soft, bluish mass of the right thenar eminence was clinically diagnosed as a hemangioma. Several months later when the lesion started rapidly enlarging the nature of the tumor was identified, and it was treated with a multidisciplinary approach that included surgical resection, chemotherapy, and radiation.