Amyotrophic lateral sclerosis (ALS) is an inexorably progressive degenerative disease that affects motor neurons. The patient with ALS is often accompanied by oral complications, such as tongue atrophy, tongue fasciculations, dysarthria, dysphagia and sialorrhea. Here, we presented a case who was diagnosed ALS with tongue atrophy, dysarthria, dysphagia and sialorrhea. We consider and arrange dental treatment and follow up.