Tracheobronchopathia osteochondroplastica (TO) is a rare disorder with unknown aetiology. We report one case of TO in a patient with non-Hodgkin pulmonary lymphoma and benign epidermal and trichylemmal cysts on the periorbital region and scalp. To the authors' knowledge, the coincidence of these processes has never been described before. The case was evaluated with conventional X-ray, computed tomography, magnetic resonance imaging, fibre-optic bronchoscopy with 2 months interval, histopathological evaluation, Tc-99 m MDP bone scintigraphy and microbiological studies including PCR testing of bronchial biopsy and lavage specimens for Mycobacterium tuberculosis. Additionally, case reports of TO in the Turkish literature are summarized.