Abstract
Ectopic ovarian tissue is an extremely rare gynecological variation. Its classification is controversial. The true origin of ectopic ovarian tissue seems to lie in an alteration in the migration of germ cells during the embryonic period. Diagnosis requires histopathological confirmation. There are about 50 published cases of ectopic ovarian tissue in the literature and almost half describe some abnormality of ectopic ovarian tissue. There are only three reports of malignant tumors in a supernumerary ovary (adenocarcinoma). We present the first reported case of clear cell carcinoma arising in an accessory ovary and also provide a review of all cases published to date.
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