WILMS' tumor accounts for about 6% of all renal malignant neoplasms. Approximately three fourths of these tumors occur in children under the age of 4 years.1This neoplasm develops insidiously and may be massive before symptoms are present. Wilms' tumor is associated with congenital anomalies, of which the most frequently noted is aniridia.2-4Other associated congenital anomalies include hemihypertrophy, pigmented nevi, hemangiomas, horseshoe kidney, duplication of the upper urinary tract, aplastic or hypoplastic kidneys, cryptorchidism, hypospadias, undescended testes, bilateral small fibrous ovaries, recurved pinna, microcephaly, mental retardation, and multiple neurofibromatosis. Associated cardiac anomalies have included tetralogy of Fallot with patent foramen ovale (one case), aortic stenosis (one case), patent ductus arteriosus (two cases), and patent foramen ovale in two patients at ages 4 and 6 years.5 The present case is of interest from the following standpoints: (1) to document an additional variety of congenital anomalies (transposition of