Tick-borne encephalitis virus (TBEV) belongs to the Flaviviridae family and Flavivirus genus. TBEV is maintained in transmission cycles between Ixodid ticks and wild mammalian hosts, particularly rodents. A wide range of animal species are also infected with TBEV by the bite of infected ticks, and TBEV infection causes fatal encephalitis in humans. TBEV is endemic widely in the Eurasian continent, and more than 10,000 cases of the disease are reported annually. In Japan, the 1st confirmed case of TBE was reported in the southern area of Hokkaido in 1993, and after 20 years, the 2nd to 4th cases were reported in Hokkaido in 2016 and 2017. Our sero-epizootiological survey indicated endemic foci of TBEV are widely distributed in Hokkaido and that those of TBEV or tick-borne flavivirus outside Hokkaido. In this review, I introduced recent topics of TBEV including newly developed diagnostic methods, epidemiology and pathogenesis of TBEV.