Progressive respiratory failure is a common modality of death in children with a terminal illness. The management of respiratory failure, and in particular symptoms of dyspnoea and musculoskeletal chest pain in children receiving palliative care, remains challenging. The emergence of palliative care paediatricians and the application of non-invasive ventilation to children with progressive respiratory failure are the two major advances in the care of children with respiratory complaints in the palliative care setting. This article outlines current approaches to palliative care in children with progressive respiratory symptoms.