[Objective] To investigate the efficacy of nephron-sparing surgery (NSS) for the treatment of renal sinus angiomyolipoma and summarize the surgical experience. [Methods] The clinical data and follow-up results of 10 cases of renal sinus angiomyolipoma treated during Sep.2014 and Feb.2022 in our hospital were retrospectively analyzed. [Results] The patients included 1 male and 9 female.The mean tumor diameter was (5.7±2.8) cm.The RENAL score was 8 in 1 case, 9 in 3 cases, 10 in 4 cases, and 11 in 2 cases.Retroperitoneal laparoscopic partial nephrectomy was conducted in 3 cases, robot-assisted laparoscopic partial nephrectomy in 3 cases, and open partial nephrectomy by transabdominal approach in 4 cases.All operations were successful.The median operation time was 225 (97-340) minutes and the median warm ischemic time was 30.5 (5-43) minutes.Two patients underwent renal vein clamping simultaneously, with clamping time of 18 and 29 minutes, respectively.The median estimated blood loss was 200(10-600) mL, and no patients received blood transfusion.The difference between postoperative and preoperative hemoglobin concentration was statistically significant [113 (94-130) g/L vs.136 (95-150) g/L, P=0.041]. The difference between the postoperative and preoperative serum creatinine level was not statistically significant [58(35-89) μmol/L vs. 62(39-77) μmol/L, P=0.722]. One case suffered lymphorrhagia (Clavien-Dindo grade Ⅰ) and recovered with conservative treatment; 1 case experienced local recurrence after a follow-up of 99 months; 9 patients recovered uneventfully without any postoperative complications. [Conclusion] NSS is an effective treatment strategy for renal sinus angiomyolipoma.Complete resection plays a key role.Due to possible recurrence, long-term follow-up is recommended.