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The Mayer-Rokitansky-Küster-Hauser (MRKH), defined as congenital hypoplasia of the structures derived from the müllerian ducts, occurs in female patients, usually with proper development and karyotype 46XX, with an incidence of 1/5,000, and is the second cause of consultation by secondary amenorrhea. It is classically divided into type I, or isolated, and type II, associated with other malformations. There have been described isolated cases of MRKH associated with the presence of adnexal tumors. Clinical case: A case of a female patient with a diagnosis of type II MRKH with the presence of adnexal tumor is presented, which according to Jacobs presents risk of malignancy; she was treated at our institution according to international clinical practice guidelines.

Tumor anexial en el síndrome de Mayer-Rokitansky-Küster- Hauser. Presentación de un caso clínico y revisión de la literatura