Babesiosis is caused by intraerythrocytic parasites that are transmitted primarily by ticks, infrequently through blood transfusion, and rarely through transplacental transmission or organ transplantation. Human babesiosis is found throughout the world, but the incidence is highest in the Northeast and upper Midwestern United States. Babesiosis has clinical features that resemble malaria and can be fatal in immunocompromised and older patients. Diagnosis is confirmed by identification of Babesia parasites on blood smear or Babesia DNA with polymerase chain reaction. Standard treatment consists of atovaquone and azithromycin or clindamycin and quinine for 7 to 10 days.