In Japan parasitic diseases have been considered to be successfully controlled in the last 30 years. However, some parasitic diseases, such as food-borne zoonoses and/or larva migrans, are emerging and/or re-emerging in Japan. Furthermore, imported parasitic diseases like malaria are also gradually increasing. Unfortunately accurate numbers of parasitic diseases other than echinococcosis, malaria, amebiasis, giardiasis, or cryptosporidiosis are obscure in Japan because of the lack of a legal registration system. Since symptoms and diagnostic imaging patterns of parasitic diseases are non-specific and have similarities with other infectious diseases or cancer, parasitic diseases are sometimes overlooked or left misdiagnosed. In this review, the current status of parasitic diseases in Japan is briefly summarized based on the analysis of the accumulated cases seen in our department. We also outline the clinical features, differential diagnosis and treatment of representative parasitic diseases for the better understanding and management of the parasitic diseases in Japan.