The translation and translation regulation of RNA in eukaryotic cells have a significant impact on cellular gene expression and maintenance of proteomic homeostasis. Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects upper and lower motor neurons and leads to muscle weakness and atrophy. More and more studies have found RNA translation abnormalities in ALS. This article provides an overview of RNA translation and regulation in eukaryotic cells under physiological and stress conditions, and explores the relationship between four different ALS-related genes and translation abnormalities, providing new ideas for the treatment of ALS.