Rationale: Pancreatic alveolar echinococcosis (AE) is an exceptionally rare disease; no more than 10 cases have been published to date. It is characterized as extensive local tissue infringement and destruction; thus, extended surgical resection, such as duodenopancreatectomy, is often needed to obtain a negative resection margin so as to improve the long-term outcome and prognosis. Patient concerns: A middle-aged Tibetan man was admitted due to a 16-year history of recurrent pain in the right upper abdomen. Magnetic resonance imaging scan showed a cystic lesion in the VI segment of his liver and several cystic lesions in the head of pancreas . Diagnoses: Pancreatic AE. Interventions: The patient adopted a preserved surgery of partial cystectomy and completely removing the content of the cyst and then soaking by hypertonic saline combined with adjuvant medical therapy of albendazole. Outcomes: The patient was monitored on a regular basis at the outpatient department; the patient is still alive and has already survived 8 years till now. Lessons: A preserved surgery combined with adjuvant medical therapy of albendazole can also contribute to a good survival outcome for AE located at the head of pancreas .
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