Spermatocytic seminoma (SCS) with sarcoma is an extremely rare testicular tumor with only 11 cases previously described in the literature. We present the 12th case of SCS with sarcoma in a 29-year-old male. SCS itself is an uncommon germ cell tumor with a relatively indolent clinical course that mostly affects males around the fifth decade of life. Sarcomatous differentiation of SCS occurs in 5% to 6% of cases and correlates with a higher possibility of metastatic disease and a poor prognosis. Clinically, this tumor manifests as a slow-growing testicular mass often with an accelerated period of secondary growth. After a concise review of the literature, we conclude that SCS with sarcoma should be treated by radical inguinal orchidectomy with strong consideration given to adjuvant chemotherapy.