Neuromuscular disorders (NMD) form a group of diseases of the peripheral nervous system with systemic manifestations, whereas the ventilatory impairment could be one of the most serious complications. The aim of this study is to describe the effects of non-invasive ventilation (NIV) associated with telemonitoring in patients with NMD. A prospective study of patients with NMD and indication for NIV due to a reduced FVC on spirometry or respiratory symptoms was carried out. These patients were followed up with periodic exams, weekly telemonitoring and constant adjustments of ventilatory parameters for 6 months. 37 patients were included, 56.7% male, average age 49.7 years (±12.6). 69.5% reported dyspnea before the indication of NIV and 10% needed medical attention. The average FVC at inclusion was 67.7 (±14.9) and 72.4 (±15.7) after 6 months, with average diurnal oxygen arterial saturation of 95.22 (±5.4) at inclusion and 95.7 (±5.1) after 6 months. Throughout the study, 4 (10.8%) patients died and 2 (5.4%) underwent tracheostomy. Between inclusion and after 6 months, according to telemonitoring, there was an increase in the average daily use in hours from 5.5 (±2.2) to 5.6 (±2.0), with a drop in leakage (L/min) of 1.9 (±6. 6) to 1.0 (±7.0) and the AHI from 3.15 (±4.9) to 2.2 (±4.3). It is concluded that diurnal oxygen arterial saturation was not a good parameter for indicating NIV, as well as FVC <70, since most patients already had respiratory manifestation. Regular follow-up of patients associated with telemonitoring showed an improvement in ventilatory parameters, maintaining stability in adherence to therapy over the 6 months.