Introduction: Renal artery pseudoaneurysm is a rare vascular lesion. Particularly, spontaneous rupture of pseudoaneurysms without predisposing events in hemodialysis patients has not been reported. We report a rare case spontaneous renal pseudoaneurysm rupture in a patient undergoing hemodialysis with Alport syndrome.   Case: A 25-year-old male with a history of end-stage renal disease visited the emergency room due to sudden severe right flank pain. He had no history of trauma or urological procedure except for a left renal biopsy to diagnose Alport syndrome 10 years ago. Diagnostic tests were performed. Contrast-enhanced computed tomography revealed right perirenal hematoma with pseudoaneurysms. Renal angiography was performed for diagnosis and treatment. On renal angiography, multiple pseudoaneurysms were observed and embolization was performed. No further aneurysms were observed after angiography.