Neurological disease may result in respiratory dysfunction; however the manifestations of respiratory dysfunction in such patients may be atypical because of wider effects of their underlying condition. In the present review we have considered separately acute neuromuscular respiratory disease (as well as aspects of respiratory muscle function relevant to intensive care), chronic neuromuscular respiratory disease, sleep related disorders, respiratory consequences of specific neurological diseases, and neurological features of respiratory disease. Approaches to specific clinical problems are discussed; in many instances this can be expedited by close cooperation with a respiratory physician. We suggest that management of respiratory dysfunction in neurological disease depends critically on three factors: firstly, knowledge of when respiratory dysfunction is likely to occur; secondly, maintaining a high index of clinical suspicion (specifically apparently vague symptoms should not be uncritically attributed to the underlying neurological condition); and, thirdly, the pursuing of appropriate investigations.