Objectives: To consider the utility of non-invasive positive pressure ventilation (NIPPV) support during percutaneous gastrostomy procedure in amyotrophic lateral sclerosis (ALS) patients with severe respiratory insufficiency and weight loss. Methods: Percutaneous gastrostomy was performed in 25 ALS patients with forced vital capacity (FVC) below 50% of predicted normal value. NIPPV was applied to all these patients during the procedure. To estimate the utility of NIPPV application during gasrtostomy tube placement, safety and procedure related complications were investigated. Results: Percutaneous endoscopic gastrostomy (PEG) was performed successfully in 21/25 patients (84%). Percutaneous radiologic gastrostomy (PRG) was performed to the rest. FVCP (predicted value of FVC) in seated position were 1,239.1 ml (32.1%) in PEG-successful group and 1,065.0 ml (26.8%) in PEG-failed group, respectively. All the patients tolerated the use of NIPPV successfully and there were no respiratory complications with the procedure. There were no major complications and procedure-related mortality in all the patients. Conclusion: NIPPV support during percutaneous gastrostomy tube placement could make the procedure possible in ALS patients with very low vital capacities