For the individual as well as for those caring for the patients, the diagnosis of amyotrophic lateral sclerosis (ALS) provides a great emotional challenge. Many factors, including existential distress, contribute to the emotional strain of patients with ALS. This study focuses on patients diagnosed with ALS and how they communicate existential issues related to meaning and guilt, relations, diagnosis and information, physical inability, and dying with dignity and respect for the person. The results of the present study indicate that (1) patients experience a number of problems, particularly in connection with physical inability, (2) the need to confide in someone is not particularly strong, (3) central for the value of life is to be respected as a person, (4) existential issues are of great importance to the patients.