Background Huntington's disease patients may ask their physician for euthanasia. The Dutch Euthanasia Act (2002) states that euthanasia and physician-assisted suicide are not punishable if the attending physician acts in accordance with criteria of due care. A review committee assesses whether a case complies with the criteria. A reconnaissance was made of cases published by the review committee. We looked into patients’ characteristics and their experienced suffering, and compared these to Parkinson's disease (PD) and Progressive Supranuclear Palsy (PSP) patients requesting euthanasia. Our clinical impression is that HD patients are influenced by their experiences with close relatives affected with HD. Method On the review committee's website, we looked up all reports concerning euthanasia in HD patients that took place between 2006–2009. We compared these to reports on PD and PSP patients (all are progressive neurological diseases with cognitive decline). Results Four reports were found concerning euthanasia in HD patients, two in PD and two in PSP patients. Both groups consisted of 3 women and 1 man. Mean age was 63 years in the HD group, and 73 years in the PD/PSP group. Disease duration in HD ranged from 5–12 years, in PD/PSP 1–4 years; all patients still lived at home. Life expectancy in the HD group was estimated months to years, in the PD/PSP group one to several weeks. Complaints/symptoms were similar in all patients. Time between first talk and euthanasia was one to several weeks in the PD/PSP group, and several months in the HD group. In all cases the due care criteria were met. Conclusion HD patients’ fear of going through the same process as close relatives plays a role in their suffering and expectations. Life expectancy for HD patients who are complete dependant may still be months-years. Upcoming admission to a nursing home is seen as a turning point. Fear of cognitive decline, of total dependency, and being unable to give meaning to life, are important ...