This chapter highlights the most important neuromuscular disorders affecting respiration, discusses their clinical characteristics and provides a guide to management. Respiratory involvement is common in many neuromuscular disorders (NMDs) to a variable degree. In most NMDs, hypoventilation is due to insufficient respiratory muscle pump and results in reduced quality of life and increased morbidity and mortality. Moreover, upper airway muscles and brain can be involved resulting in obstructive sleep apnea, central sleep apnea or central hypoventilation syndrome. Especially in congenital neuromuscular diseases with early disease onset, skeletal deformities reduce thoracic compliance with resulting restrictive ventilatory pattern. In some neuromuscular disorders, more than one system can be affected with the need for an individual diagnostic and therapeutic approach. Intensive care and long-term management of these conditions are discussed.