Horseshoe kidney (HSK) is a congenital kidney anomaly that urologists frequently encounter for different reasons such as obstruction, stones, and malignancy.1 Xanthogranulomatous pyelonephritis (XGP) is a rare and potentially harmful diagnosis as it may lead to serious complications such as sepsis, renal failure, and potentially life-threatening conditions.2 A combination of HSK and XGP is rarely encountered and creates a challenge to urologists in the prospect of diagnosis and management. Few articles were published showing the feasibility of laparoscopic heminephrectomy in such a situation.3 Therefore, we present a case of XGP and HSK treated with modified transperitoneal laparoscopic heminephrectomy.