〔Abstract〕 Objective To investigate the clinicopathologic features, diagnosis and treatment and prognosis of uterine leiomyoma with fumarate hydratase (FH) deficient. Methods The clinical data of 26 patients with uterine leiomyoma with FH deficient admitted to Fujian Maternal and Child Health Hospital from November 2018 to February 2022 were retrospectively analyzed. Results The mean age of patients was (38.92 ± 7.42) years. The clinical manifestations were menstrual changes (30.77 %) and abdominal pain during menstruation (19.23 %). There were 16 cases (61.53 %) with imaging revealed degeneration of uterine tumor (or) rich blood flow. 17 cases (65.38 %) were multiple fibroids during surgery, and 9 cases (34.62 %) were single fibroids. All patients were followed up for an average of (14.81 ± 7.91) months after surgery, and all survived, 5 of them (19.23 %) with fibroid recurrence. The postoperative recurrence rate of patients with different ages, family history of uterine fibroids and postoperative recurrence rate of Ki-67 index patients, the differences were statistically significant (P < 0.05). Conclusion FH deficient uterine leiomyoma is the sentinel manifestations of hereditary leiomyomatosis renal cell carcinoma (HLRCC). It has the characterized of young age of onset, multiple fibroids and large size, imaging of uterine mass degeneration and rich blood flow, intraoperative freezing and pathological findings of cell, and are prone to relapse, and can be accompanied by renal cancer. Clinical attention should be focused on patients with risk factors such as age < 42 years, family history of uterine fibroids, and Ki-67 index ≥ 5 %.