Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne infectious disease caused by the SFTS virus (SFTSV), a novel phlebovirus reported to be endemic to China in 2011. In Japan, the first SFTS patient was identified during the autumn of 2012; since then, over 100 SFTS patients have been reported. The SFTSV has been identified throughout Japan over the past two years; however, SFTS patients are specifically localized to western Japan. The clinical symptoms of SFTS include fever, thrombocytopenia, leukocytopenia, gastrointestinal symptoms, and various other symptoms, including muscular symptoms, neurological abnormalities, and coagulopathy. SFTS is often accompanied by hemophagocytic syndrome. The histopathological findings are characterized by necrotizing lymphadenitis, with infiltration of the virus-infected cells to the local lymph nodes. Pathophysiological analyses of SFTS include studies regarding the kinetics of cytokine production and immune responses in patients with SFTS and in SFTSV-infection animal models. This article aimed to survey the history of SFTS in Japan and to review the clinical, epidemiological, and virological aspects of SFTS and SFTSV infection.