Progressive neuromuscular disease requires increasing degrees of respiratory support to sustain life. Each step from intermittent to continuous—and noninvasive to invasive—ventilation requires thoughtful consideration based on the goals of the patient and family, and the inherent benefits and burdens of the treatment. Tracheostomy, in particular, should not be viewed as an inevitable next step when less permanent or invasive methods prove insufficient. Like other modes of respiratory support, tracheostomy may represent a bridge to recovery of pulmonary function, or a stabilizing action in the hope that novel therapies may prove beneficial. In other situations, tracheostomy represents a destination therapy, necessitating consideration of the implications of chronic mechanical ventilation. Institutional, social, and financial considerations may affect decisions related to tracheostomy, as may implicit bias regarding quality of life. The complexity of such care and decisions highlight the need for optimal palliative care throughout the patient’s life.