Renal cell carcinoma on imaging unveiled as a primary Ewing’s sarcoma

Abstract Background Ewing sarcoma represents a spectrum of aggressive malignancies with the poor outcome. Primary renal Ewing sarcoma is rare and accounts for less than 1% of renal masses. Case presentation We present a case of a 45-year-old male presented in OPD with complaint of right flank pain and hematuria. He underwent a contrast-enhanced CT abdomen which depicted a right renal mass with liver lesions. He underwent Robotic Right Radical Nephrectomy and a pathological diagnosis of Ewing Sarcoma was made after which he was started on chemotherapy. Conclusions Owing to presentation in younger age group with poor prognosis, an integrated analysis including radiological imaging, histopathology, and immune-histological staining is essential for early detection of renal Ewing sarcoma.