We reported a case of 1 year and 3 month old baby with huge right renal mass, hypospadias, bilateral, undescended testes, umbilical hernia and right inguinal. The patients underwent transabdominal right radical nephrectomy; repair of umbilical hernia; right orchidopexy and repair of the right internal ring. No uterus or ovaries were seen in the pelvic cavity during exploration. Karyotyping was 46XY and serum testosterone level was normal. Histopathology of the kidney revealed congenital mesoblastic nephroma (CMN). This were aims to determine whether there is a relationship between the CMN and the intersex disorder.