We report a rare case of peripheral adenoid cystic carcinoma of the lung, showing unusual pathological and clinical features, namely rapid growth, local aggressive behaviour, huge tumour size, no endobronchial component or submucosal infiltration, and a rapidly progressive clinical course. Extensive surgery resulted in considerable palliation of symptoms, but not in prolonged survival. The reported case emphasizes the malignant potential of peripheral adenoid cystic carcinoma of the lung.