Canine babesiosis is a tick-borne disease caused by protozoal haemoparasites of different Babesia species. Babesiosis is one of the most important globally extended and quickly spreading tick-borne infections of dogs. This comprehensive review gives an in-depth overview of Babesia species currently identifi ed in dogs together with relevant vector tick species and their geographical distribution, life cycle and transmission of parasite. The main mechanisms in the pathogenesis of babesiosis are described and elucidated by recent literature overview. As Babesia infection causes a disease with very variable clinical manifestations, special attention is given to clinical signs, laboratory features and clinicopathological fi ndings. The diagnosis of canine babesiosis by microscopy, serological and molecular methods is reviewed, together with recent advances in mass spectrometry based assays. Accurate detection and species recognition are important for the selection of the appropriate therapy, monitoring and prediction of the outcome of the disease. Finally, guidelines for the treatment and prevention of canine babesiosis are given.