Abstract Background: Uterine leiomyoma is the most common benign tumor in the female reproductive system. Fumarate hydratase (FH)-deficient leiomyoma is a rare subtype characterized by distinctive histopathological and immunophenotypic features and may be associated with hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome. Case description: A 35-year-old Han Chinese woman, gravida 1 para 1, was admitted in 2021 because of multiple uterine leiomyomas.