Tick-borne encephalitis (TBE) is an infectious zoonotic disease, moving from Central Europe to other countries and still rare in Italy. The disease, produced by the European subtype virus, typically takes a biphasic course with neurological disorders of different severity during its second phase. We report the first three TBE cases in Friuli Venezia Giulia (FVG), characterised by extremely variable clinical features. Knowledge of these different presentations will assist physicians in increasing their level of attention to TBE also in this region, where no cases of TBE had been reported in the past, despite the fact that it borders countries with high prevalence of the infection.