In a retrospective review of the clinical course of five patients with nontrauma-related bilateral diaphragmatic paralysis, we found that the diagnosis is generally delayed (median delay: two years) in the presence of moderate to severe respiratory insufficiencies. Orthopnea out of proportion to the underlying cardiopulmonary status and thoracoabdominal paradoxus in the supine position are the clues to the diagnosis. Pulmonary function tests showed a typical restrictive ventilatory defect, and arterial blood gas showed chronic respiratory acidosis. Failure to recognize the diaphragmatic impairment led to cardiopulmonary decompensations such as cor pulmonale and acute respiratory failure. However, with the institution of appropriate therapy, adverse effects resolved and all five patients are alive and continue to lead independent lives. We conclude that nontrauma-related bilateral diaphragmatic paralysis is an underrecognized and underdiagnosed condition. The results also indicate that early diagnosis and treatment can minimize morbidity and mortality, and that excellent long-term, good-quality survival can be expected.