Ewing’s sarcoma/primitive neuroectodermal tumors (ES/PNETs) are a group of malignant tumors with varying degrees of neuroectodermal differentiation. Once regarded as distinct entities, ES and PNET are now recognized as a same family of tumors based on similar characteristics. Although they may develop in any tissue organs, tumors originating from small intestine are exceedingly rare. A case of 9-year-old girl presenting with abdominal pain, melena, and iron deficiency anemia is reported here. Image work up showed multiple masses in small bowel and omentum with disseminated peritoneal seeding nodules, implying lymphoma as first impression. Pathological reports from explorative diagnostic laparoscopic biopsy showed tumors comprising of densely packed, small round proliferating cells. Immunohistochemically CD99 and CD56 were positive and EWS-FLI1 translocation was observed by dual color, break apart rearrangement fluorescence in situ hybridization suggesting intraabdominal ES/PNET. Neoadjuvant chemotherapy comprising of VDC (vincristine, doxorubicin, cyclophosphamide) alternating every 3 weeks with IE (ifosfamide, etoposide) was performed. Tumor burden decreased gradually during five consecutive cycles of systemic chemotherapy with no evidence of any distant metastasis. Segmental resection of jejunum, where the tumor was located, was conducted. Subsequently, the patient underwent adjuvant chemotherapy. Considering wide abdominal dissemination at the time of diagnosis, high dose chemotherapy followed by autologous peripheral blood stem cell transplantation is currently on schedule. This is the first pediatric case of ES/PNET found in small intestine in Korea.