Background: Hydatid disease is a parasitic infectious disease caused by Echinococcus granulosus. The parasite can form cysts in any part of the body with the liver and lung being the most commonly involved organs. It can rarely occur in other organs like the muscle, bone, pericardium, myocardium, spleen, spine, and neck. Para-vertebral hydatid cyst is very rare and can present with a variable clinical presentation. Surgical excision is the treatment modality of choice and accepted to be curative with a very low recurrence. Case presentation: We here present a case of primary hydatid cyst in the para-vertebral space in sub-occipital area of the neck who presented with complaints of pain and swelling in the right posterior-lateral side of the neck. X-ray cervical spine revealed suspicious lesion in the sub-occipital area. Magnetic resonance imaging (MRI) was suggestive of multiloculated cystic lesion. There were no such cysts found in peritoneal viscera or any other organ. Patient was planned for surgery and the cyst was excised. Histopathological examination confirmed the hydatid disease. Post-operatively, the patient was put on albendazole and patient's symptoms disappeared after surgery. Conclusion: Cysts in any part of the body should be evaluated for hydatid disease especially in the endemic areas. Radiological imaging and serology are important for diagnosis, and surgical excision is the best modality of treatment.