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Prion diseases, of which Creutzfeldt-Jakob disease is the most common, are fatal neurodegenerative disorders and are often rapidly progressive. They are associated with a significant palliative care burden for patients and families, ranging from prognostic uncertainty to complex symptom management to caregiver distress. Healthcare professionals face unique pressures when caring for these patients, which can include a lack of familiarity with this rare diagnosis and rapidly evolving symptom needs due to accelerated clinical deterioration. We convened a multidisciplinary panel of experts from around the UK, including palliative care doctors, general practitioners, physician and nurse specialists in prion diseases, and a lived experience representative to compile practical, consensus-based recommendations for managing prion diseases, much of which can also be applied to other rapidly progressive dementias. In this article, we examine the available evidence base for managing various aspects of prion diseases. Where evidence is limited, we suggest best practices informed by decades of our collective experiences.