RESUMO
BACKGROUND: Extraskeletal Ewing sarcoma (EES) is a rare and highly malignant small round cell tumor associated with a poor clinical outcome. Ewing sarcoma (ES) involving the stomach is an uncommon presentation and can be easily confused with other small round cell tumors. We herein present a rare case of ES involving the gastric area. CASE SUMMARY: We report a case of gastric ES in a 19-year-old female patient who initially presented with a complaint of a tender epigastric mass for 5 d. Contrast-enhanced abdominal computed tomography revealed a soft-tissue-density mass with a diameter of 8.5 cm between the liver and stomach; the mass was connected to the gastric antrum. Then, the mass was surgically excised completely. Upon histopathological, immunophenotype and molecular analysis, the mass was identified to be a primary gastric ES. CONCLUSION: EES is an aggressive tumor with poor prognosis. Therefore, early diagnosis and timely intervention are essential for a good prognosis. It is imperative for us to raise awareness about this rare tumor. Surgical resection is still the best treatment option.