Introduction: Glomus tumors are rare benign mesenchymal neoplasms accounting for only 2% of all types of soft tissue tumors. Commonly located in the peripheral soft tissues, they are most frequently encountered in the subungual areas of fingers and toes, and very rarely in visceral organs due to the absence of glomus bodies. To date, 22 cases of primary renal glomus tumors have been described in the literature, of which 17 benign, with no evidence of recurrence or metastasis, three cases of malignant glomus tumor, and two cases with uncertain malignant potential. Case report: We report the 18th case of a benign glomus tumor of the kidney in a 49-year-old female patient, presenting the microscopic appearance (round, uniform cells with indistinct borders, scant finely granular eosinophilic cytoplasm, round nuclei lacking prominent nucleoli, arranged in solid sheets, accompanied by slit-like vascular spaces), the immunohistochemical profile (tumor cells showed immunoreactivity for smooth muscle actin, vimentin, as well as for CD34; they were negative for AE1/AE3, desmin, HMB-45, S-100 protein, renin, and chromogranin), and the differential diagnosis of this rare entity (juxtaglomerular tumor, angiomyolipoma, hemangioma, epithelioid leyomioma, solitary fibrous tumor, carcinoid tumor, and paraganglioma). Conclusion: Primary renal glomus tumors are rare tumors that radiologically can mimic other mesenchymal renal neoplasm. Accurate diagnosis is based on the microscopic appearance and especially the characteristic immunophenotype.