Telocytes (TCs) represent a new distinct type of interstitial cells found in many organs, including lungs. TCs are mainly defined by a small cellular body from which arise very long (hundreds of micrometers) extensions named telopodes. During the last years, TCs were characterized in respect with their microRNA profiles, gene features and proteome signatures. Also, the ultrastructural 3D configuration was further elucidated by the aid of the FIB-SEM technology. TCs are able to communicate by homo- and heterocellular contacts with neighboring cells and are also able to transfer genetic information and signaling molecules to influence other cells by means of extracellular vesicle release. However, the exact function of lung TCs remains unclear. Here, we review the potential significance of TCs in the pathogenesis of pulmonary diseases. We will also discuss some future possibilities for targeting TCs as a potential therapeutic strategy.