Respiratory distress resulting from a neuromuscular disorder is often misdiagnosed and not managed properly until development of severe respiratory failure. Amyotrophic lateral sclerosis (ALS) is a rare but archetypical motor neuron disease. Most cases manifest as weakness, muscular atrophy, and progressive paralysis of the limbs. Late stage of the disease is characterized by respiratory failure due to respiratory muscle weakness. In a few cases, 3% of patients, respiratory failure is the initial presentation, preceding other symptoms. We report on a case of ALS accompanied by acute respiratory failure and weaning failure, with a review of the literature.