Malakoplakia is an unusual acquired granulomatous disease that can affect many systems including urogenital tract. It presents a huge diagnostic challenge as it can mimic malignancy. We report a 55-year diabetic woman who presented with history of macroscopic hematuria and right flank pain. On investigations, ultrasound of kidney, ureter and bladder (KUB) showed right hydronephroureter, and CT KUB showed right moderate hydronephroureter and right ureteric stone. Endoscopic examination revealed multiple white plaques involving urinary bladder and right ureter. The diagnosis of malakoplakia was based on microscopic findings that are specific for its diagnosis.