Melanosis is defined as an abnormal or excessive deposition of melanin within cells and/or tissues. It typically presents as a cutaneous or buccal mucosal lesion, but rare cases of bladder melanosis have also been documented. Melanosis of the urinary bladder is typically considered a benign condition, but it has also been described in association with malignant melanoma and urothelial carcinoma.We report the cases of 2 patients who presented with melanosis of the urinary bladder. One patient presented with melanosis of the urinary bladder together with urothelial dysplasia. Melanosis was incidentally identified during a cystoscopy for ureteral stones. A second patient presented with hematuria and was found to have a muscle invasive urothelial carcinoma with focal small nested morphology together with melanosis. We also present a literature review of the bladder melanosis and an overview of other bladder melanocytic lesions, which include primary and metastatic melanoma and blue nevus.Initial evaluation for bladder melanosis should include cystoscopy and upper urinary tract imaging. Biopsy is essential to establish the diagnosis and rule out associated malignancy.