Liver fluke infection remains a major neglected zoonosis with public health implications, mainly in East Asia and Africa. Most patients are initially asymptomatic and thus rarely diagnosed at early exposure. Biliary tract inflammation and carcinogenesis are known to be closely related; persistent infection by these parasites increases the incidence of cholangiocarcinoma (CCA) in these endemic areas. Clonorchis sinensis and Opisthorchis viverrini are classified as Group 1 carcinogens by the International Agency for Research on Cancer, considered carcinogenic to humans (Bouvard et al., Lancet Oncol 10:321–322, 2009). Preventing exposure and adequate early treatment may contribute to reducing the incidence of the deadly disease. Herein, we overview the epidemiology and characteristics of liver fluke infection, describing treatment from a surgical perspective.