Amyotrophic lateral sclerosis (ALS) is a devastating terminal neurodegenerative disease with a highly predictable clinical course such that palliative care should begin at or soon after diagnosis. The outcome is certain in most cases. The only medication approved for treatment in the United States, riluzole, extends life by about 2 months. Virtually all skeletal muscles eventually are affected. Multiple problems require a multidisciplinary approach including aggressive symptomatic management, rehabilitation to maintain motor function, nutritional and respiratory support, augmentative communication devices, and psychological support for both patients and families because family members so often play a central role in management and care. Social, bioethical, and financial issues as well as advance directives should be addressed long before enteral feeding or assistive ventilatory support might be considered. Goals of care should be assessed on an ongoing basis. Presenting the unusual case of a patient with ALS who is also a prominent neurologist specializing in ALS, we enumerate issues in management and palliative care applicable to ALS but also to other fatal, progressive neurologic diseases such as Huntington's chorea and late-stage Parkinson disease.