Abstract Horseshoe kidney is the most common congenital renal fusion anomaly. It is more prone to complications such as pelviureteric junction obstruction and renal stones. Spontaneous rupture of the urinary collecting with urinoma has never been reported in a horseshoe kidney in literature. Spontaneous rupture of the renal pelvis is a rare event even in orthotopic kidneys and can be due to obstructing calculus. Spontaneous rupture in the horseshoe kidney can mimic acute abdominal conditions. We report a case of a 29-year-old man who presented in the emergency department with severe mid-abdominal pain. Contrast-enhanced computed tomography (CT) evaluation of the abdomen showed contrast extravasation outside the right pelvicalyceal system with pelviureteric junction obstruction in a horseshoe kidney with extensive collection in the subhepatic, subdiaphragmatic, and right pleural region. The patient underwent a double-J stent placement which was followed by complete healing of the urinary leak and underwent definitive open pyeloplasty after 3 months. Diagnosis and therapeutic challenges in this rare case are being discussed.
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