Inflammatory myofibroblastic tumor (IMT) is a rare occurrence most commonly affecting the pediatric population. It is considered a diagnostic challenge because of nonspecific clinical symptoms and radiologic presentations. IMT is a soft-tissue tumor that can occur throughout the body but is most commonly found in the lung. This article discusses the case of a 10-year-old girl who presented to our hospital with abnormal chest radiograph. Imaging revealed a heterogenous mass in the superior aspect of the left lower lobe. It was found incidentally when the patient presented to the emergency department. Surgical resection proved a diagnostic and therapeutic measure confirming diagnosis of IMT. The tumor has potential to cause illness either by its rare malignant potential or by mass effect. Because lesions are often asymptomatic with a predilection for pediatric patients, cases describing appropriate management are essential. IMT is a rare tumor of borderline malignant potential. IMT of the lung may invade hilar structures, the mediastinum, and the pleura. Lesions range in diameter from 1.5 to 14 cm. Etiology of the tumor is unclear but may include immune response to infection, trauma, or surgery. Affected patients may present with cough and shortness of breath but are often asymptomatic. Histology is unique but not specific. Immunohistochemistry can differentiate the tumor, by staining for overexpression
of the anaplastic lymphoma kinase oncogene. In conclusion, surgical resection is the preferred diagnostic and therapeutic regimen for IMT. The majority of IMTs follow a benign course, but exceptions qualify as low-grade malignancies. As a result, it is important to identify, treat, and maintain vigilance. This case encompasses presentation, diagnosis, and treatment of pulmonary IMT and highlights best practices in addressing IMT for adolescent patients.