Inflammatory myofibroblastic tumors (IMTs) are a group of soft tissue neoplasms with a predilection for the lungs and abdominopelvic cavity, characterized by a mixture of fasciitis-like, compact spindle cells, hypocellular fibrous histologic patterns, and distinctive molecular features. Due to their unspecified symptoms and non-specific radiologic presentation, the histopathologic and immunohistochemical analysis of a biopsy specimen is crucial for the diagnosis. We present a case of a 30-year-old man with intermittent hemoptysis diagnosed with a pulmonary IMT. We aim to review the literature regarding its definition, clinical findings, diagnosis, treatment, and prognosis. The treatment for an IMT is based on its location and extent, including complete surgical resection, which has a good prognosis compared to corticosteroids, chemotherapy, radiotherapy, and non-steroidal immunomodulation in patients who are not good surgical candidates. Further investigative studies with larger sample sizes and longer meticulous follow-ups are needed to demonstrate this neoplastic disease's natural history and find appropriate management for it.