Purpose: We aim to evaluate the correlation of neurocognitive findings with pulmonary functions in Myotonic dystrophy type 1 (DM1).Materials and Methods: This is an observational prospective (between 2013-2015 years) cross-sectional single center study at Neurology and Chest clinics of Cukurova University. Mini mental state examination, forward and backward digit span, verbal fluency (semantic and lexical), clock drawing, verbal and visual memory, pulmonar function tests, arterial blood gas samples were performed to DM1 patients and control group. The presence of correlation between neurocognitive tests and pulmonary function, arterial blood gas samples were researched in DM1. Results: There were significant differences between DM1 (10 male, 14 female) and control group (12 male, 9 female) on verbal fluency, clock drawing, forward and backward digit span tests. There was a positive correlation (p<0.05) between verbal fluency and respiratory function tests (total lung capacity) in DM1.Conclusion: In the present study; we encountered a positive correlation between total lung capacity and verbal fluency in DM1. Pulmonary function tests and neurocognitive assessment should be performed concomitantly in DM1 patients. This provides early detection and treatment of cognitive impairment and loss of pulmonary functions.