Bulbous urethra involved in perineal extraskeletal myxoid chondrosarcoma in a child

Extraskeletal myxoid chondrosarcoma is a rare soft-tissue sarcoma that usually occurs in deep soft tissues, especially those of the proximal extremities and limb girdles, but is rare in children. We present an unusual case of a tumor arising in the perineum and involving the bulbous urethra in a 21-month-old boy. The patient was treated with surgical excision and urethroplasty followed by combination chemotherapy.