BACKGROUND Granular cell tumor is usually a benign tumor, generally believed to be of neural origin, most commonly affecting the tongue and skin. Although it can present in any part of the body, the bladder is a rare location, with only 16 cases found in the English-language literature. CASE We report the case of a 54-year-old woman with hematuria who had a solid tumor in the posterior wall of the bladder. Histological study of the samples obtained by transurethral resection revealed a granular cell tumor, confirmed by immunohistochemical techniques. CONCLUSION Granular cell tumors of the bladder are rare and generally benign but frequently present macroscopic features resembling those of urothelial carcinoma. The similarity can lead to an erroneous clinical diagnosis and unnecessary, aggressive treatment. A careful histopathological assessment is essential for an accurate diagnosis.