We report the first case of an endobronchial perineurioma, a rare benign neoplasm typically occurring in soft tissue. A 53-year-old nonsmoking female presented with a three-month history of persistent bronchitis. A CT scan followed by bronchoscopy demonstrated an endobronchial lesion involving the left mainstem bronchus. Removal of the lesion by bronchoscopy was accomplished. The tumor was composed of bland spindle cells in a variably collagenized stroma. These cells had long cytoplasmic processes. No mitotic activity or necrosis was observed. Neoplastic cells were immunoreactive for epithelial membrane antigen (EMA), CD34, and claudin-1. Smooth muscle actin (SMA), desmin, and S-100 immunostains were all negative. Based on the morphologic appearance and immunophenotype, a diagnosis of perineurioma was rendered.