Primary adenoid cystic carcinoma (ACC) of the peripheral lung is a rare entity. Here we report two cases of primary ACC. Case 1 is an 84-year-old male with a past-medical history of cecal cancer presented with a 10 mm left upper lung nodule. Case 2 is a 40-year-old female who presented with 30 mm right upper lobe. Intraoperative (Case 1) and pre-operative (Case 2) histopathologic and cytologic diagnoses were consistent with a primary peripheral lung ACC. An upper lobectomy±mediastinal lymph node dissection was performed and immunohistochemical staining with thyroid transcription factor (TTF)-1, c-KIT and MYB on the excision specimen confirmed our diagnosis. carcinoma in the peripheral lung: a cytological, histopathological and immunohistochemical report of two cases. Pathol Discov. 2015; 3:3. http://dx.