A 79-year-old man presented to our outpatient clinic with symptoms of dysphagia, dysarthria, and muscle atrophy of the trunk and upper extremities. These symptoms were gradually progressive, and he had lost substantial weight-20 kg in 2 years. One month later, he was admitted due to dehydration and received tube feeding. The presence of "split hand" suggested amyotrophic lateral sclerosis (ALS). Finally, the patient was diagnosed with ALS by two neurologists. When elderly patients present with progressive dysphagia and muscle atrophy, especially with "split hands," ALS should be included as a differential diagnosis.