Previous studies have demonstrated that domiciliary ventilatory support improves survival, quality of life and cognitive function in motor neuron disease (MND). These benefits are partly attributed to better sleep quality and less hypoxia. In a retrospective analysis we compared the effect of non-invasive (NPPV, n = 52), tracheostomy (TPPV, n = 23) and no ventilation (n = 43) on sleep and survival in MND patients over a seven-year period. The TPPV and NPPV groups had more sleep arousals (AI, p = 0.024), more respiratory events (p = 0.001) and more time asleep with an oxygen saturation less than 90% (%TST with SpO2<90%, p = 0.01), than those who were not ventilated. After treatment with TPPV or NPPV, the percentage of rapid eye movement sleep increased (p<0.001) and the %TST with Sp02<90% (p = 0.006) and AI (p = 0.001) decreased. Improvements were larger and more consistent with NPPV. The median survival of those who used TPPV was 41 months, NPPV 32 months and of those not ventilated was 25 months, significantly different four years (p = 0.0497) after symptom onset. In this retrospective cohort of MND patients, ventilation, particularly NPPV, markedly improved sleep and conferred a modest survival advantage.