Introduction: Ewing sarcoma most commonly arises in bones but rarely presents in extraskeletal locations. We report one such case arising from the jejunum.
Case presentation: A 67-year-old woman presented with acute-onset, right lower quadrant pain. Computed tomography results showed a large mass within the midjejunum with pneumoperitoneum. Surgical excision was performed, and an extraskeletal Ewing sarcoma of the jejunum was suspected histologically. The diagnosis was confirmed with fluorescence in situ hybridization studies.
Conclusion: This case emphasizes the importance of recognizing this rare presentation in the small intestine to broaden the differential diagnosis of adult intraabdominal tumors.