Juxtaglomerular cell tumor (JCT) of the kidney is a rare benign renal neoplasm. Four cases of JCT of the kidney have been diagnosed and treated surgically in our hospital from January 2005 to August 2008. The clinical features, laboratory examination, imaging examination, pathological results and electron microscopy examination were analyzed, and a review of the literature was summarized. Three patients underwent open partial nephrectomy, and one patient underwent laparoscopic partial nephrectomy. Pathological examination confirmed the final diagnosis of JCT of the kidney. The blood pressure, potassium, renin and aldosterone remained within the normal range after surgery. JCT of the kidney should be kept in mind because they represent a surgically curable cause of secondary hypertension. Nephron-sparing surgery is recommended.