We present a case of unusual localization of inflammatory fibroblastic tumor in the terminal ileum, cecum, and ascending colon in a 7-year-old child. Segmental resection of the terminal ileum, cecum, and ascending colon with a tumor mass up to 6 cm in diameter was performed. Pathohistological examination of biopsy specimen was performed on routine hematoxylin-eosin sections, as well as immunohistochemically with primary antibodies to CD3, CD20, CD68, factor VIII, vimentin, smooth muscle actin, desmin, cytokeratin and S-100 protein, and k and l light chains. The tumor was composed of highly vascularized tissue with interlacing fascicles of elongated spindle cells admixed with plasma cells, histiocytes, lymphocytes, and eosinophils. The diagnosis of inflammatory myofibroblastic tumor was confirmed by immunohistochemistry. Inflammatory myofibroblastic tumor cannot be distinguished clinically from highly malignant neoplasm or some other conditions. Surgical resection and careful pathohistological analysis are needed, and a long-term follow-up is recommended.