Amyotrophic lateral sclerosis Is an unusual pattern of distal muscular atrophy with permanent bulbar sign. Vocal fold paralysis and velopharyngeal insufficiency(VPI) due to soft palate paralysis Is occasionally associated with distal muscular atrophy. Recently we experienced a case of amyotrophic lateral sclerosis whose symptom was initially expressed with hoarseness and VPI. So we report a case with review of literature.