Introduction: Pancreatic hydatid cyst (PHC), a rare parasitic infectious disease, is caused by the larvae of Echinococus granulosus. Common human organs affected by the hydatid cyst are the liver and lunge, but it rarely involves other organs such as the pancreas. Case Presentation: A rare case is reported of a patient with a pancreatic head hydatid cyst. The result of the patient's preliminary serological surveys was negative and the pancreatic lesion was suspected to be a pseudocyst or cystic pancreatic neoplasms. Following complete excision of the lesion, the pathological analysis of the surgical specimen confirmed hydatid disease. Conclusions: Pancreatic hydatid cysts may be confused with more common lesions of the pancreas such as pseudocysts and cystic pancreatic neoplasms. Physicians should always consider this disease in the differential diagnosis, especially for patients from endemic areas.