Background: Primary renal Ewing’s sarcoma (EWS) is unusual in adults. Diagnosis is usually made on histopathological examinationation supported by immunohistochemistry. We aimed to review the clinical and histopathological data of patients with renal EWS and review the available literature. Methods: we reviewed our database from January 2015 to june 2024 to include all the cases of primary EWS of the kidney. Later on we performed systematic literature review to identify all the relevant series on renal EWS. Results: A total of three patients including two men and one woman were managed during the above mentioned period. Out of these two patients had venous tumour thrombus and none had metastasis at diagnosis. All patients underwent elective radical nephrectomy, two patients received adjuvant chemotherapy while none was subjected to radiotherapy (RT). On Immunohistochemistry (IHC), CD99 and NKX2.2 were positive in all the patients. All patients are alive without disease at a follow up ranging from 6 months to 25 months. In our review 17 studies were included, 29.88% of the patients had metastatic disease and 15.6 % had locally advanced disease at diagnosis. Average overall survival was 33.5 months. CD99 and FLI1 were positive in 95.6% and 85.7%, respectively. Conclusion: Primary renal EWS remains a pathological diagnosis and IHC has an important place in its diagnosis. Locally advanced and metastatic disease is common at diagnosis leading to overall poor survival.