Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with no definitive cure. This review summarizes the current pharmacotherapeutic strategies, focusing on approved agents such as riluzole and edaravone, while critically evaluating their clinical efficacy and limitations. It also explores emerging drugs under development based on diverse pathogenic hypotheses, highlighting ongoing global clinical trials. Furthermore, the review discusses the potential of personalized and combination therapies. This work aims to provide a comprehensive framework for understanding ALS pharmacotherapy.