The crossed ureteral ectopia with solitary kideny is a rare urological anomaly. To our knowledge, only about 30 cases have been reported in literature. We had experienced of a 4 month old girl, who was admitted due to abnormal urethral discharge and recurrent UTI for 2 months. Cystic-like lesion over right abdominal cavity was detected by sonogram. The CT scan disclosed a small right kidney, located normally, and compensatory hypertrophy of the left side kidney. It was also found that right abdomen filling with dilated ureter. The cystoscopic examination revealed, with difficulty, the right ureteral orifice opening into the contrateral side of urethra, located in left anterior-lateral wall proximal to the external sphincter muscle of bladder neck. The exploratory laparotomy disclosed the small right kidney (2.5 X 2.0 X 2.0 cm^3) with normal blood vesseles. The ureter was found huge dialated and tortuous. Nephroureterectoy was performed. The pathology revealed dysplastic change of the right kidney, and thickening with fibrotic change of the ureter. The UTI was improved after surgery.