Primary yolk sac (endodermal sinus) tumor of the prostate is extremely
rare with only 2 cases reported in the literature. We describe a case
of primary yolk sac tumor of the prostate in a man with Klinefelter's
syndrome. Treatment included 4 courses of combination chemotherapy fo
llowed by retroperitoneal lymph node dissection, cystoprostatectomy an
d ileal conduit urinary diversion. We review the association of Klinef
elter's syndrome with extragonadal germ cell tumor along with the mana
gement of this rare disease.