A 7-month-old child presented with imperforate anus, penoscrotal hypospadias and transposition, and a midline mucosa-lined perineal mass. At surgery the mass was found to be supplied by the median sacral artery. It was excised and the anorectal malformation was repaired by posterior sagittal anorectoplasty. Histologically the mass revealed well-differentiated colonic tissue. The final diagnosis was well-differentiated sacrococcygeal teratoma in association with anorectal malformation.