We report a rare case of granular cell tumor in the scrotum. Granular cell tumors are soft-tissue neoplasms originating from Schwann cells that rarely affect male external genitalia. They are essentially benign; therefore, the treatment is complete excision of the lesion. Although never previously reported in the male external genitalia, malignant variants exist in 2% of cases. Because the clinical presentation is not specific, the diagnosis of malignant granular cell tumors can be made only by the pathologist. To our knowledge, only 5 other cases in the scrotum and 19 cases described in the penis have been reported.