Abstract
Objective: To address the Mayer-Rokitansky-Kuster-Hauser Syndrome (SMRKH) and the dysgerminoma germ cell tumor, analyzing the association between these two pathologies, as well as their complications. In addition to discussing the treatments offered to both, showing the patient's management and the surgical approach performed in the case described. Details of the case: Patient, 20 years old, clinical picture of primary amenorrhea associated with progressive increase in abdominal volume and pelvic pain. Examination showed a solid and heterogeneous expansive lesion in the right ovary, concomitantly with findings of dysgenesis of the lower portion of the uterus and of the vagina, equivalent to SMRKH. After indicated surgical treatment, she revealed pT1N1 staging ovary dysgerminoma, FIG IIIA1. Follow-up with chemotherapy and subsequent follow-up, with no signs of recurrence to date. The collection of information was authorized by the patient through the informed consent form (ICF). Final considerations: After reviewing the comparative literature to the case presented, it is concluded that the association between Mayer-Rokitansky-Kuster-Hauser Syndrome (SMRKH) and the germ cell ovarian tumor, of the desgerminoma type, is rare. And the approach with complete cytoreductive surgery for ovarian cancer associated with chemotherapy, along with psychological support to patients is a possible approach.