Abstract
A 10-year-old female with atrial septal defect presented with bilateral ovarian mature cystic teratomas. Three spleniculi adjacent to the main spleen were noticed while the endoscopic surgery. Two years later a heart tumor was discovered which led to death. Pathologic examination of samples of the mass proved it to be a liposarcoma. The unusual combination of atrial septal defect, f-anisosplenia (female, congenital heart disease, multiple unevenly-sized spleens, left lung type pulmonary isomerism), bilateral ovarian mature cystic teratomas and liposarcoma of the heart appears to be a unique presentation.
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