Abstract
Malakoplakia is a rare inflammatory disorder primarily affecting the urinary tract, characterized by granulomatous reactions and Michaelis-Gutmann bodies. It typically presents with nonspecific urinary symptoms, mimicking malignancies radiologically and histologically, thereby complicating diagnosis. This case describes an uncommon presentation in an 18-year-old male with hematuria and dysuria, initially misdiagnosed as a urothelial neoplasm. Accurate diagnosis required extensive histopathological analysis, revealing classic malakoplakia features. Management included endoscopic resection and ureteral stenting. This emphasizes the diagnostic challenges posed by malakoplakia, the importance of careful multidisciplinary evaluation, detailed histopathology for correct identification, and the need for early intervention and follow-up to avoid renal complications.