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If familial hereditary tumor is suspected, diagnosis and treatment should always be performed considering the presence of familial hereditary tumors irrespective of whether genetic testing is performed. © 2020 The Authors. Clinical Case Reports published by John Wiley Sons Ltd.Adenocarcinomas of intestinal type arising in mature cystic teratoma of ovary (MCT) are extremely rare and remain a diagnostic dilemma because of its similarities with MCT. Serum tumor markers CEA and SCC and also MRI may help in the preoperat