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We reported a case of a GCT of the urinary bladder and review the literature. A 23-year-old female presented with dysuria that had lasted for the previous 6 months. MRI revealed a 3 × 2.5 cm global mass in the anterior wall of urinary bladder. Cystoscopy showed a semispherical tumor approximately 3 cm in diameter that was covered with normal bladder mucosa and extended from the bladder neck to the anterior wall of the bladder. The patient underwent transurethral resection of the tumor. Histological examination and immunohistochemical staining showed a granular cell tumor (GCT). There were no features suggesting a malignant phenotype. On 6 months follow-up, the patient has remained free of bladder recurrence. We recommend careful pathologic assessment for establishing the appropriate diagnosis and either a conservative or aggressivesurgical treatment for benign or localized malignant GCT of the urinary bladder, respectively.