Inflammatory myofibroblastic tumor (IMT) is reported virtually in every anatomic location of the body, but most cases are commonly identified in the mesentery and omentum. The etiology of this tumor is unclear with many suggestions of viral, inflammatory, or oncogenic mutational factors that establish it as a clonal neoplasm. Clinical and laboratory workup, including roentgenography, is not usually helpful to reach a pre- or intraoperative diagnosis. Histopathology and immunohistochemistry of the resected specimen provides a definitive answer by the exclusion of a close clinical differential diagnosis of gastrointestinal stromal tumor and many lookalikes. Complete surgical excision with clear margins is the mainstay of treatment. Rare cases have been seen involving the appendix. To the best of our knowledge, only 11 confirmed cases of purely appendiceal IMT have been published in the literature to date.