Abstract
Rhabdoid tumor is a highly aggressive sarcoma found in young children that occurs in the kidney, central nervous system and soft tissue sites. Rarely, it presents in the fetus or neonate and is associated with a dismal prognosis. We report a case of a 28-week gestation fetus presenting with hydrops fetalis who died soon after delivery, found at autopsy to have a rhabdoid tumor of the thoracic cavity with placental metastases and provide a review of the literature of congenital sarcomas.