In recent years nutrition assessment and management in amyotrophic lateral sclerosis (ALS) have drawn increased attention. Frequent evaluation of nutrition status is warranted in ALS, given the common occurrence of dysphagia and hypermetabolism and varying disease progression rates. Nutrition management includes dietary and swallow strategies, possible gastrostomy tube placement, and recommendations for vitamin and mineral supplementation. Strategies to assess and optimize nutrition status and prolong survival in ALS patients are reviewed with recommendations based on current research.