Anoplocephala perfoliata is the commonest tapeworm parasite of horses and is incriminated as a significant cause of clinical disease (e.g., ileocaecal intussusception, caeco-caecal intussusception and/or caecal perforation), particularly in horses chronically infected with large numbers of worms. The high prevalence (approximately 20-80%) of the parasite in some countries suggests an increased risk of clinical cases. In spite of research, there is still a paucity of information regarding the pathogenesis of the disease, the epidemiology of the parasite in different geographical regions and there are significant limitations with the diagnosis of infection. The present article provides an account of the biology, epidemiology and pathogenic effects of A. perfoliata, the diagnosis of infection and treatment. It highlights some gaps in knowledge of the parasite and the disease it causes, and suggests opportunities for future research and prospects for improved diagnosis, prevention and control.