AIM: In this study, we aimed to investigate the possible role of endotrophin, a profibrotic byproduct of collagen VI, in the complex process of fibrosis development in the disease group with pulmonary fibrosis among interstitial lung diseases. MATERIAL AND METHOD: When the patients’ participation in the study were completed, smoking or alcohol drinking conditions, and family history were recorded. Their weights and heights were recorded and body mass index (BMI) was calculated. In every patient, Spirometry with bronchodilator testing, determination of single-breath DLCO, and plethysmographic measurement of thoracic gas volume and airway resistance were performed. Blood samples were obtained for the inflammation markers such as sedimentation rate, C-reactive protein (CRP), complete blood count, liver and renal function tests, and lactate dehydrogenase levels. Serum endotrophin levels were measured in all patients. RESULTS: Thirty-five patients with interstitial lung disease who were having pulmonary fibrosis, 35 patients with interstitial lung disease without pulmonary fibrosis, and 20 control patients without any signs or symptoms of interstitial lung disease were included in the study. Age distribution was similar between groups. The fibrotic ILD group was more commonly smoker or ex-smoker compared with the non-fibrotic ILD patients or control cases. Fibrotic ILD patients were leaner, having significantly decreased total lung capacity, diffusion capacity, and higher LDH levels. In the comparison of the 3 study groups regarding the endotrophin levels, there was a significant difference between groups. The fibrotic and non-fibrotic patient groups were compared for the Endotrophin levels and the difference was also significant. However, there was not any significant difference regarding the endotrophin levels between control cases and non-fibrotic ILD patients. Smoked cigarette pocket x year showed a significant positive correlation and DLCO % and KCO % showed a significant negative correlation with the endotrophin levels. CONCLUSION: Serum endotrophin levels significantly increase in fibrotic ILD patients compared with the non-fibrotic ILD patients and control cases. Endotrophin may be suggested as a diagnostic marker in fibrotic interstitial lung diseases.