A case of an intrarenal teratoma in a 5-mo-old infant has been presented with the pertinent roentgenographic and pathologic features. The lesion was originally diagnosed as a Wilms' tumor and chemotherapy was initiated before pathologic confirmation. Though extremely rare, this tumor must also be considered in the differential diagnosis of an abdominal mass in the first year of life. The kidney represents one of the less common sites of extragonadal germ cell tumors. A review of the literature pertaining to the distribution, clinical, and pathologic features of extragonadal germ cell tumors in infancy is presented.