Recent respiratory developments have altered the management of children with severe neuromuscular disease, and in some cases changed the natural history of the conditions. These developments include improvements in diagnosis; a greater availability of ventilatory support techniques, including noninvasive modes; and better functional scoring systems that enable global muscle function and the impact of treatment to be evaluated. Greater choices can make for more difficult decision making, and the ability to prolong survival brings with it opportunities, but also ethical dilemmas. Clearer outcome information and assessment of the benefits and burdens of ventilatory support for the child and family should aid decision making. In many conditions there is no relationship between functional ability, the need for ventilatory support, and quality of life. Noninvasive ventilation combined with cough assistance is usually preferred to invasive ventilation and is likely to reduce respiratory morbidity. In situations where noninvasive ventilation does not extend survival, it may have a role in palliating symptoms and allowing the child to be cared for at home.