A premature neonate (gestational age 32-week) weighing 1800 gr with no penis was born from a 27 yrs old mother with no special disease and a healthy boy. The parents were healthy with no family relation. No drug was taken during gestation and no certain chemical proximity was noted. The mother had once unknown induced abortion history. The only mentioned point in pregnancy period sonography has been Oligopolyhydramnios. The baby's Karyotyping was XY. Examination revealed his respiratory distress. Checking his head and neck, the ears were located lower than their normal position and saddle nose was observed. There were featured chest, distended belly and higher located navel and the liver was touched 3 cm below the ribs. Via examining genitals, phallus has not been seen. Scrotum was normal and contained gonad with normal dimensions and consistency. Sacrum was short but anus was normal with normal meconium excretion. In the lower limbs, two sided clubfoot was observed (). Echocardiography reported mild tricuspid heart insufficiency and small openness of patent ducts arteriosus. In abdominal sonography, massive bladder with increased thick wall has been seen. The kidneys were severely atrophic with several cysts (multicystic bladder). In cystography, the contrast media reflux into urachus and bilateral ureter was observed. Urethra was short leading to distal rectum but no obvious opening was spotted (). Due to no urination, the patients underwent cystostomy. During the operation, biopsy was taken from gonads, which reported normal testes. 2nd day after birth, blood urea, creatinine and potassium increased and on the 25th postnatal day, the baby died because of clinical signs of renal failure.