Crimean Congo hemorrhagic fever (CCHF) has the most extensive geographic distribution of the medically significant tick-borne viruses. Its causative agent is a negative-sense, single-stranded RNA virus belonging to the family Bunyaviridae, genus Nairovirus. The virus can be transmitted mainly through direct contact with blood or tissues from infected livestock or through bites of Hyalomma ticks. Moreover, nosocomial and community outbreaks have been already described. Although in Greece serologic evidence of the virus has been observed, no case of CCHF has been reported until 2008; however, on June 2008 a case of CCHF was reported in Greece and phylogenetic analysis showed that the causative agent of CCHF was a virus strain similar to other strains detected or isolated in the Balkan Peninsula, Russia and Turkey which are associated with severe and sometimes fatal disease in humans. This case identification raised many concerns on the emerging potential and the changing epidemiology of CCHF. The present article reviews on the epidemiological and clinical features of CCHF; moreover, prevention and control strategies are being described in detail.