HTLV-1 is a significant global health problem but remains largely confined to endemic areas and risk groups. However, increasing migration may mean that the virus will be encountered more frequently in areas traditionally thought of as virtually free of HTLV-1. In this review we discuss the epidemiology, transmission, clinical features, diagnosis and treatment of HTLV-1, focussing specifically on the neurological manifestations. We highlight the circumstances in which HTLV-1 should be suspected and outline the current understanding of HTLV-1-associated myelopathy and other neurological presentations.