The poor prognosis of amyotrophic lateral sclerosis (ALS) makes palliative care a challenge for the neurologist. Most of the disabilities from progressive disease can be effectively relieved by symptomatic treatment. Prognosis and treatment options should be openly discussed with patient and relatives. Adequate assistance and palliative treatment in the terminal phase are of paramount importance. Unfortunately, training in communication skills for young doctors and evidence-based recommendations for palliative care are insufficient at present. In addition, new data from a randomized study question the concept of "health-related quality of life" and favor an individualized approach to the definition of quality of life in ALS.