Barw Barw Medical Journal 2960-1959 3 3 2025 06 18 Renal Ewing Sarcoma: A Case Report and Literature Review 51 55 10.58742/bmj.v3i3.186 eng Rebaz M. Ali Oncology Department, Hiwa Cancer Hospital, Shorsh Street, Sulaymaniyah, Iraq. rebaz.ali@gmail.com Bnar Sardar Saida Department of Nephrology, Shar Teaching Hospital, Sulaymaniyah, Iraq. bnar.saida@gmail.com Saman S. Fakhralddin College of Medicine, University of Sulaimani, Madam Mitterrand Street, Sulaymaniyah, Iraq. saman.fakhralddin@univsul.edu.iq Ari M. Abdullah Department of Pathology, Sulaymaniyah Teaching Hospital, Sulaymaniyah, Iraq. ariabdullah1978@gmail.com Hadeel Adnan Yasseen College of Medicine, University of Sulaimani, Madam Mitterrand Street, Sulaymaniyah, Iraq. hadeel.yasseen@gmail.com Dilan S. Hiwa Scientific Affairs Department, Smart Health Tower, Madam Mitterrand Street, Sulaymaniyah, Iraq. dilan.sarmad.hiwa@gmail.com Marwan A. Ghafoor School of Health Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia. marwan.ghafoor@gmail.com Jalil Salih Ali Department of Radiotherapy, Zhianawa Cancer Center, Sulaymaniyah, Iraq. jalil.ali@gmail.com Shano M. Ali Hiwa Cancer Hospital, Shorsh Street, Sulaymaniyah, Iraq. shano.ali@gmail.com Soran H. Tahir College of Medicine, University of Sulaimani, Madam Mitterrand Street, Sulaymaniyah, Iraq. soran.tahir@univsul.edu.iq Rawa M. Ali Hospital for Treatment of Victims of Chemical Weapons, Halabja, Iraq. rawa.ali@gmail.com Zhino Noori Hussein Department of Urology, Sulaymaniyah Surgical Teaching Hospital, Sulaymaniyah, Iraq. zhino.hussein@gmail.com Abdullah K. Ghafour Scientific Affairs Department, Smart Health Tower, Madam Mitterrand Street, Sulaymaniyah, Iraq. abdullah.ghafour@gmail.com 2025 04 25 Introduction Primary renal Ewing sarcoma is an extremely rare and aggressive tumor, representing less than 1% of all renal tumors. This case report contributes valuable insights into the challenges of diagnosing and managing this rare malignancy, particularly when it presents with atypical symptoms. Case presentation A 30-year-old female presented with intermittent grassy green-colored urine, later turning into red, with a headache and no abdominal pain. Clinical examination revealed elevated blood pressure. Imaging studies, including ultrasound and computed tomography scans, identified a large, heterogeneous mass in the left kidney with invasion into the renal vein and lymph nodes, leading to a staging of T3N1M0. The patient underwent a left radical nephrectomy, with pathology confirming a Grade 2 primary Ewing sarcoma / primitive neuroectodermal tumor of the kidney. Despite aggressive treatment with adjuvant chemotherapy, stable metastatic deposits persisted, indicating ongoing active disease. Literature review Ewing sarcoma typically occurs in bones but can occasionally arise in solid organs such as the kidney. Most patients present with non-specific symptoms, and the disease often remains undiagnosed until it has metastasized. Current treatment involves multimodal therapy, including surgery and chemotherapy, but prognosis remains poor, especially in cases with metastasis. Conclusion This case underscores the complexity of diagnosing and treating primary renal Ewing sarcoma. Persistent metastasis despite treatment highlights the need for vigilant monitoring. Further genetic profiling could enhance understanding and management of this rare condition.