Inflammatory myofibroblastic tumors are uncommon tumors, which present as solitary masses in the pulmonary parenchyma. Tracheal involvement by these tumors is extremely rare and can be misdiagnosed as asthma. The closest histologic differential diagnoses are IgG4-related sclerosing pseudotumors, which are differentiated by IgG4 positivity. Fifty percent of inflammatory myofibroblastic tumors are positive for anaplastic lymphoma kinase gene rearrangements. The treatment modality of choice is surgical resection with therapeutic bronchoscopy reserved for patients presenting with acute airway obstruction or in those unwilling for surgery. New and upcoming treatments include anaplastic lymphoma kinase inhibitors like crizotinib.