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Introduction: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with an inexorably progressive course which leads to a progressive neuromuscular weakness. Weight loss is one of the major bad prognostic factors in ALS. The placement of percutaneous endoscopic gastrostomy (PEG) is of paramount importance in patients with dysphagia to improve the disease outcomes, although some fear exists regarding the possible ventilatory complications during the procedure. The aim of this study was to evaluate the safety and effectiveness of PEG tube insertion under non-invasive ventilation (NIV) in patients with ALS and severe ventilatory impairment., Methods: A retrospective study of all consecutive PEGs placed in our department from May 2011 to January 2018 in patients with ALS was performed. The procedure was performed under non-invasive positive-pressure ventilation for ventilatory support., Results: We included 59 patients with ALS with severe ventilatory impairment, 58% were female, with a mean age of 67.2 ± 10.1 years and a median follow-up of 6 [2-15] months. The main indication for PEG placement was dysphagia (98%). The median time for PEG tube insertion since the established diagnosis of ALS was 12 [6-25] months and 4 [2-18] months since the beginning of bulbar symptoms. The majority of the patients had placed a 20-Fr PEG (63%) and under mild sedation with midazolam (80%), all under NIV. There were no immediate complications during and after the procedure (no episodes of aspiration or orotracheal intubation) and mortality., Conclusion: The placement of PEG is a very important procedure in patients with ALS and severe ventilatory impairment. The interdisciplinary department collaboration permitted the placement of PEG under NIV, in a safe and effective procedure in this special population., Competing Interests: The authors disclose no possible conflicts of interest., (Copyright © 2021 by Sociedade Portuguesa de Gastrenterologia Published by S. Karger AG, Basel.)