Uterine leiomyomas, also known as uterine fibroids, are the most common pelvic tumors in women of reproductive age, affecting more than 70% women globally and serving as the most common indication for hysterectomy [1]. Fumarate hydratase (FH)-deficient uterine leiomyomas represent a rare but clinically significant subset of uterine smooth muscle tumors, as they comprise just 1.6% of fibroids despite being associated with hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome [1,2]. The gene discussed is FH; the disease is uterine corpus leiomyoma.