Endobronchial inflammatory pseudotumor (EIP) is an uncommon, benign entity in the central airways, frequently causing acute respiratory insufficiency by occluding the trachea or main-stem bronchi. Surgical resection of the tumor is a treatment option; however, it is associated with high morbidity. We recently encountered the case of a 24-year-old woman with EIP in the central airways that was successfully treated with nonsurgical multimodality approach using a combination of steroid therapy, radiotherapy, and repetitive bronchoscopic electrocauterizations. A review of the literature is also provided. We used a PubMed search to find case reports of inflammatory pseudotumor in the central airways that had been written in English and published since 1975. Reports of peripheral lesion of the subsegmental bronchi or lung parenchyma were excluded. Twenty-seven reports describing 30 cases of EIP in the central airway were identified. Nine cases were treated without surgical resection and 4 cases, including our case, were documented to have residual extratracheal extension of the tumor, even after bronchoscopic intervention. However, recurrent respiratory distress or intratracheal regrowth was not documented in these cases during a follow-up period of 1.5 to 9 years. Although the EIP in our patient was confirmed histologically to be benign, recanalization of airway and control of regrowth of the tumor by a combination of radiotherapy, steroid, and bronchoscopic intervention was proven to be an acceptable therapeutic option.