Malakoplakia, an anecdotal reactive granulomatous lesion, is a rare pathologic entity but relatively more common in genitourinary tracts. Here we report a case of malakoplakia in the urinary bladder in systemic sclerosis. The patient was a 66-year-old female treated with long-term corticosteroid and cyclophosphamide. She presented with gross hematuria, and cystoscopy and biopsy revealed malakoplakia. There was no tumor and the stains for infectious organism were all negative. To the best of our knowledge, this is the first case report of malakoplakia in a systemic sclerosis patient.