Although rare in developed countries, hydatid disease is a serious parasitic infection in endemic areas. Spinal disease most commonly involves the thoracic region, and involvement of the upper cervical spine is extremely rare. In this report, a case of hydatid disease involving the craniocervical junction is presented, along with a review of the literature. A 44-year-old man presented with the complaint of neck pain. The results of his physical and neurological examinations were within normal ranges, except for pain exacerbated by neck motion and spasm of the cervical musculature. Magnetic resonance imaging demonstrated a cystic lesion involving the odontoid process and body of C1 and C2, with thin and regular cyst walls and cyst contents similar in intensity to that of cerebrospinal fluid. The results of serological tests performed with the suspected diagnosis of hydatid disease were positive. The patient initially underwent surgery to provide stabilization of the craniocervical junction, using autogenous bone graft and sublaminar wiring from the occiput to C3 via a posterior approach. The cyst was approached via a transoral route, using a U-shaped pharyngeal incision. There were no neurological deficits after surgery. Postoperative magnetic resonance imaging scans confirmed complete excision of the cyst, and the patient was discharged on the 12th postoperative day. He received six cycles of albendazole treatment, each consisting of 28 days with an intervening drug-free period of 2 weeks. Magnetic resonance imaging scans performed 1 year after surgery revealed the patient was still disease-free. Hydatid disease should be considered in the differential diagnosis of spinal cord compression, especially in endemic areas. Although the chance of obtaining a cure is unlikely, radical surgery coupled with antihelminthic therapy seems to provide long-lasting relief.