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A primary yolk sac (endodermal sinus) tumor of the prostate is described in a 40-year-old male. This is the second documented case of a yolk sac tumor occurring in the prostate gland. Treatment included surgical removal and subsequent combination chemotherapy. Four months following diagnosis, the patient died of complications. At autopsy, no residual tumor was detected. The role of combination chemotherapy as potentially curative therapy in the treatment of extragonadal yolk sac tumors is discussed.
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