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Squamous cell carcinoma arising from a mature teratoma of the ovary is rare. Diagnosis is usually made after surgery, although some elements allow presumption, including patient age, tumor size and tumor marker dosage. Treatment is a big challenge because of the rarity and aggressiveness of this entity of ovarian cancer. We report the case of a 50-year-old woman with squamous cell carcinoma arising from a mature teratoma of the right ovary. Through this observation, we review the various clinical, radiological, pathological, therapeutic and prognostic aspects of this rare form of ovarian cancer. [ABSTRACT FROM AUTHOR]