The authors report on an 11-year-old boy with retroperitoneal alveolar rhabdomyosarcoma at the aortoiliac bifurcation. The tumor was resected en bloc together with the infrarenal abdominal aorta and the inferior vena cava. The aortic defect was repaired successfully with a Y-shaped Dacron graft. The authors conclude that tumor resection with aortic reconstruction can be a choice for unresectable retroperitoneal tumors in children. Copyright (C) 1998 by W.B. Saunders Company.