Hydatid disease (Echinococcosis) is a common parasitic infection caused by Echinococcus granulosus mainly in sheep-raising areas of the world. Liver, lungs and brain are the predominantly involved organs. However, 0.5-1% of the hydatid disease involves the spine and in 90% of the cases it is confined to the bone and the epidural space. Although intramedullary involvement is extremely rare, in this report, we present a 55-year-old female patient who was diagnosed with a cervical intramedullary hydatid cyst during magnetic resonance imaging of the cervical vertebrae. Accordingly, we imply that particularly in endemic areas, hydatid cyst disease should be kept in mind for the differential diagnosis of spinal mass lesions.