We report a case of bilateral multicystic kidney and left ureteropelvic junction (UPJ) obstruction in a female infant. She was diagnosed to have severe cystic disease from 20 weeks of gestation. After birth with cesarean section, she was transferred to the neonatal intensive care unit (NICU) of our hospital. One day after birth, she was referred to our department for progression of azotemia. We placed a nephrostomy catheter into the largest renal cyst, but it did not work. One week later, we placed another nephrostomy catheter into another cystic lesion inside of the first one. It worked well for nine months and azotemia was improved. At the age of nine months her upper urinary tract was reconstructed by pyeloplasty. We observed peristalsis of ureter and first urination from bladder.