Introduction : Horseshoe kidney (HSK) is type of congenital anomalies of the kidneys and urinary tracts (CAKUT), which fusion anomalies happened in early embryonic period partially. One-third of the patients with horseshoe kidney are usually asymptomatic and diagnosed incidentally. It can be presented with other renal and extrarenal abnormalities. Here we report a case of horseshoe kidney in infant presenting as recurrent urinary tract infections with severe hydronephrosis. Case Presentation: A 10-months old boy with multiple congenital anomalies admitted with a history of recurrent urinary tract infection for 3-month and progressive abdominal enlargement. His physical examination revealed an abdominal distention, no-tender, dullness on percussion, a palpable mass on the right flank, and normal bowel sound. The genitalia examination revealed penile-type hypospadia and bilateral undescended testis. Further examination with a contrast computed tomography scan showed HSK with severe right hydronephrosis with thin parenchyma due to UPJO. The heminephrectomy procedure was performed successfully. There is no recurrent UTI and no deterioration in renal function after 3 years of evaluation. Conclusion: It is important to investigate renal abnormalities in patients with recurrent UTI. HSK is one of the prevalent findings. Therefore, proper diagnostic investigation tailored to the clinical presentation in a stepwise approach aiming for prompt and optimal patient care is essential.