Introduction:Coexistence of horseshoe kidney and ureterocele abnormalities in one patient is a complex and rare condition. The role of heredity in this disease has not yet been proven. Moreover, its diagnosis requires various paraclinical methods. Case Presentation: A middle-aged male was known to have horseshoe kidney, bilateral ureteroceles and a stone, 1 cm in diameter, in his right ureter. He also had severe hydronephrosis in his left kidney, whereas mild hydronephrosis was detected in his right kidney. Unlike other similar cases, we performed a transverse incision on the ureterocele with no surgery on the horseshoe kidney. Similar cases to date have been treated with partial nephrectomy. Urinary complications and flank pain did not occur during the one-year follow up. Conclusions: Precise evaluation of patients with one renal abnormality could lead to detection of other associated anomalies. Application of minimally invasive procedures such as ureteroscopic incision is highly recommended in ureterocele treatment.