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© The Author(s) 2020.We report an instance of a 47-year-old feminine, with powerful preoperative medical and radiological suspicious of uterine leiomyosarcoma whom underwent a complete abdominal hysterectomy. Despite the final histology concluded as harmless uterine leiomyoma, the increasing loss of fumarate hydratase appearance of the same specimen still place her at an increased risk of getting genetic leiomyomatosis and renal cellular carcinoma problem. Intraoperatively, an obstetric