Objective To investigate the value of CT urine road image formation on urinary system congenital abnormality. Methods 30 cases with urinary system congenital abnormality in our hospital from January 2014 to January 2016 were selected and given CT scan, enhancement scan, volume reconstruction. Analyze the congenital anomalies diseases of urinary system. Results In renal malformations diseases of urinary system, 12 cases ectopic kidney, 6 cases solitary kidney, 3 cases renal improper rotation (2 cases discoid kidney, 1 case horseshoe kidney), 6 cases cystic dysplasia kidney, 3 cases fused kidney, 17 cases double kidney with duplicate ureters (13 cases unilateral double kidney with duplicate ureters, 4 cases bilateral double kidney with duplicate ureters). Conclusion In the treatment of 30 cases with urinary system congenital abnormality, CT scan, enhancement scan and volume reconstruction can factually show mutual relation and anatomical structure of lesion. It can lower the missed diagnosis and misdiagnosis rate by large degree and provide guiding significance on clinic treatment.