Tumors of the epididymis, both primary and secondary, and whether benign or malignant, are very rare. Adenomatoid tumors and leiomyoma are the most frequently diagnosed benign tumors of the epididymis. In a review of the American and European literature, leiomyoma was the second most common neoplasm of the epididymis, representing 6% of primary epididymal tumors. A case of leiomyoma of the epididymis in a 53-year-old patient is reported. The patient presented with a 4-year history of a painless mass in the left scrotum. There were no bother some symptoms except gradual enlargement of the tumor. On surgical exploration, the mass was found to be smooth and well-demarcated. A fresh frozen section showed a benign lesion, and conservative excision of the tumor was performed without any difficulty. The leiomyoma was definitively diagnosed by subsequent histopathologic analysis. The details of this rare case are reported herein with a review of the medical literature.