Abstract: Objective Tostudy the clinicopathologic features of juxtaglomerular cell tumor (JGCT), and investigate its diagnostic clues and prognosis. Methods Four cases with juxtaglomerular cell tumor admittedtodepartment of pathology, Chinese PLA General Hospital from 2012 to 2014 were selected. The macroscopy, microscopy and immunohistochemistry were investigated, and clinical data and relatedliteratures were reviewed. Results The JGCT cases included3 females and 1 male with the age of 44, 27, 30, 70 years. Hypertension, hyperreninemia and hyperaldosteronismwere foundin 4 cases. Hypokalemia were detected in two cases. Two cases received radical nephrectomy and two cases underwent pertial nephrectomy. Tumors were located in the renal cortex with the diameter of 2 to 5 cm and fibrous capsule was found. Microscopically, the tumor cells grew in sheets predominantly and consisted of polygonal and spindled with eosinophilic cytoplasm, but adenoid or papillary pattern could also be seen. Numerous mast cells and hyalinized thick-walled blood vessels could also be seen. One case showed chrysanthemum growth pattern, and tumor had invasive growth with infiltrating renal pelvis and round fat. Immunohistochemically, all JGCTs were strongly and diffusely positive for vimentin and Syn. Three cases stained for CD34, and two cases stained for CK (tubule), Actin and S-100. All cases showed various Ki67 index (1% to 10%), but were negative for SMA and CgA. All of them were followed up for 1 to 24 months. The blood pressure and aldosterone maintained in normal range and there were no tumor progress and metastasis. Conclusion JGCT should be considered as rare and neuroendocrine tumor with special function. Most of them have benign biological behavior, while some individual cases showing invasive growth.