The variable clinical course of Amyotrophic Lateral Sclerosis (ALS) confronts the clinician, the patient and caregivers with many ethical challenges from the moment of breaking the news of the diagnosis and throughout the relentlessly progressive trajectory of the disease. Each patient faces the prospect of life-threatening bulbar and respiratory muscle dysfunction that may ensue soon after disease onset or after months or years of progressive weakness. This reality eventually forces the patient to choose life extension via gastrostomy tube insertion, mechanical ventilation or both or to forego these treatments in favor of terminal palliative care. Faced with these prospects some patients contemplate voluntary cessation of food and water, physician assisted suicide or euthanasia. Depending upon the presence and severity of frontotemporal dementia (FTD) related to frontotemporal lobar degeneration (FTLD) the capacity to make these forced choices may be compromised. Clinicians caring for ALS patients should appreciate and communicate the significance of life threatening symptoms, monitor capacity for decision making, anticipate and manage multiple possible end of life scenarios, and aggressively manage symptoms.