Risk of malignant transformation in a dysgenetic gonad is high. This is a report of a 23 year-old female who presented with symptoms and signs of ovarian neoplasm which was histologically confirmed as embryonal carcinoma. She had features of Turner's syndrome and was found to have XO chromosomal constitution. Embryonal carcinoma arising in a dysgenetic gonad is uncommon. It is even rarer when it arises in a patient with pure 45 XO. The following is a case report which highlights the role of chemotherapy and surgery in the management. It includes a literature review on the clinical features, genetic variants and malignant transformation in dysgenetic gonad. The role of prophylactic removal of dysgenetic gonad is discussed.
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