Objective To explore the diagnostic value of spiral CT urography (CTU) for double renal pelvis ureter malformation. Methods 68 cases of patients with double renal pelvis ureter malformation treated in our hospital from September 2013 to August 2017 were selected for the study and were given spiral CTU and ultrasound. The surgical outcomes were used as standards to compare the detection results. Results 64 cases were accurately detected by spiral CTU with double renal pelvis ureter malformation, including 24 cases of simple left double renal pelvis ureter malformation, 22 cases of simple right double renal pelvis ureter malformation, 3 cases of simple bilateral double renal pelvis ureteral malformation, 7 cases of double renal pelvis ureter malformation with renal calculi, 5 cases of double renal pelvis ureteral malformation with ureteral calculi, 2 cases of right ureteral malformation with ureteral cyst and 1 case of left ureteral malformation with ectopic ureter opening. Another 1 case was misdiagnosed and 3 cases were undiagnosed. The accurate detection rate of spiral CTU was significantly higher than that of ultrasound in the detection of double renal pelvis ureter malformation (94.12% vs 79.41%) (P<0.05). Conclusion CTU has certain diagnostic value for double renal pelvis ureter malformation.