The concept of assisted ventilation in the home setting has greatly progressed as a routine practice. This technique was used from 1990 to 2000 in 16 children cared for at our center. Ten children had neuromuscular disease (infantile spinal amyotrophy 6, Duchenne myopathy 3, and mitochondrial myopathy 1) or other conditions including central hypoventilation (n = 2), traumatic tetraplegia (n = 2), encephalopathy with chronic bronchitis (n = 1) and bronchopulmonary dysplasia (n = 1). Only 5 children had a tracheotomy, the others were treated successfully with non-invasive ventilatory assistance. Initialization of non-invasive ventilatory assistance had been planned before development of respiratory failure in 4 of the 11 children, but generally was indicated after an episode of acute respiratory distress. Home ventilation, particularly with non-invasive assistance is a reliable method for long-term treatment of chronic alveolar hypoventilation in children. The appropriate time for initiating this therapy should be better defined.