A 33-year-old male complained of poor vision and visited the ophthalmology department of our hospital. Right iridoncus suspected as a metastatic nodule, was found. A systemic examination was done. Abdominal computed tomographic (CT) scan showed an enhanced mass in the right scrotum, 15 mm in diameter and enlargement of iliac lymph nodes. Chest CT scan demonstrated bilateral enlargement of mediastinal lymph nodes and enlargement of lymph nodes above the collarbone. Since malignant lymphoma or testicular malignancy was suspected, right orchiectomy was planned. A hard and nodular mass at the head of the epididymis, completely separated from the testis, was recognized. Only open biopsy was done, because no malignancy was found by the intraoperative biopsy. Pathological findings of the epididymal tissue revealed non-caseating epithelioid cell granulomas. Sarcoidosis was diagnosed based on pathological findings and systemic evaluations. Steroid ocular instillation therapy was started. To the best of our knowledge, this is the 18th report of epididymis sarcoidosis in Japan.