Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease of unknown etiology, which most commonly affects men, smokers, aged from 20 to 40. It is diagnosed by histological analysis of material obtained by lung biopsy, with immunohistochemical proving of Langerhans cells. The aim of this research is to determine pathohistological characteristics of PLCH and analyzing demographic, clinical and radiological parameters. Retrospective analysis of medical data for 13 patients, proven for PLCH at Institute for Pulmonary diseases of Vojvodina in period of fifteen years. PLCH was found at 9 (69.3%) women and at 4 (30.7%) men, average age 34.7 years. Main clinical symptoms were cough (76.9%) and chest pain (61.5%). Out of 13 patients, 11 (84.6%) were smokers. In most cases PLCH histologically corresponded to the cellular phase of the disease (46.1%), proliferative phase was present at 5 (38.4%), and the fibrotic phase at 2 (15.5%) patients. Immunohistochemically, Langerhans’ cells were positive for presence of CD1a and S-100 antigens in all 13 of analyzed cases, while CD68 antigen was positive in 6 patients. In 6 patients (46.2%) there was disease regression, and at 7 (53.8%) patients the disease progressed despite the applied therapy. In our research, PHLC was more common in younger females, smokers with cough and chest pain. At most of the patients, histologically disease was in the cellular phase. Langerhans cells were positive to presence of CD1a and S100 antigens in all 13 patients. At more than half of the patients the disease progresses despite the applied therapy.