We report about a two and a half year old girl with a primary yolk sac tumo
r (YST) of the heart, located in the interventricular septum. The girl was
admitted to the hospital with a systolic cardiac murmur. Echocardiography r
evealed a smoothly surfaced tumor which based on the interventricular septu
m below the aortic valve and filled out more than half of the left ventricu
lar cavity. Diagnosis of a yolk sac tumor was established on a frozen secti
on of a biopsy taken at surgery and confirmed by the examination of the res
ected material. Excessively elevated serum alpha-fetoprotein (AFP) levels c
ontributed to this diagnosis. Follow up examinations of the patient includi
ng computed tomography (CT) of head, thorax and abdomen, did not indicate a
n extracardiac tumor manifestation. We found no previous report of a primar
y intracardial yolk sac tumor in the literature.