[Objective] To explore the efficacy and safety of the surgical resection and differential diagnosis of rare benign tumor, reninoma, so as to provide reference for clinical diagnosis and treatment. [Methods] A retrospective analysis was made on 4 patients with reninoma in Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology combined with the data of previous literatures. [Results] Except for patient 4, who was asymptomatic, the rest of the patients had hypertension and electrolyte imbalance. Patient 1 was admitted to hospital twice with adrenocortical adenoma. No renal mass was found on the first visit, and the right kidney abduction mass was indicated during the re-examination due to persistent hypertension. Patient 2 was misdiagnosed with kidney cancer at the beginning; patient 3 had been neglected due to pregnancy and negative imaging results, and then underwent imaging examination again to show a parenchymal mass in the right kidney due to persistent elevation of hypertension, dizziness and headache. Patient 4 had non-functioning reninoma with no clinical manifestations. Patient 1 underwent left adrenal adenoma resection and right partial nephrectomy successively, and patients 2—4 underwent partial nephrectomy. The postoperative pathological results of all patients showed reninoma. The patients' blood pressure, serum potassium, plasma renin and aldosterone concentrations returned to normal after surgery.Except for patient 3, who was in shock after surgery, the rest recovered well. [Conclusion] Reninoma is a rare benign renal tumor with rapid progression and a high risk of complications.Nephron-sparing surgery has a satisfactory outcome and a good prognosis.