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For patients with motor neuron disease (MND), the final 12 months of life can be a tumultuous period, with rapid and significant losses in function and independence, regular contact with the health system and carer stress. The aim of this article is to provide an outline of the challenges encountered during the last 12 months of life and the role of the specialised multidisciplinary team in managing the challenges that may arise. While MND remains rare overall, it is likely that most general practitioners (GPs) will encounter at least one patient with MND during their career. An understanding of the complexity of this group of diseases, including management in the terminal phase, is important given the GP is a valuable member of the multidisciplinary team.