Zoonotic babesiosis has received increased attention recently, due mainly to the interest in tick-borne zoonotic diseases generated by the emergence of Lyme borreliosis and to increased awareness of diagnostic and treatment difficulties associated with co-infection cases. The vast majority of European cases have been caused by Babesia divergens in splenectomised patients, and although rare, this disease is very dangerous, requiring aggressive treatment. The use of atovaquone, a recently developed anti-protozoan agent for human treatment, may be considered in future cases. Most human babesiosis caused by B. microti have occurred in the north-eastern states of the USA and can affect spleen-intact as well as asplenic patients. The majority of infections are subclinical or follow a mild chronic course, but dangerous acute infections can occur in immunocompromised patients. The role of B. microti in apparently unresponsive cases of Lyme borreliosis and treatment of co-infections require further investigation. The zoonotic potential of B. microti in Europe is still unresolved, but the vector competence of Ixodes ricinus for at least some European (and American) strains has been demonstrated.