BACKGROUND Alveolar hydatid disease (AHD) is a rare and severe parasitic infection caused by the larval stage of the fox tapeworm, Echinococcus multilocularis. AHD mainly involves the liver, and although it may extend progressively to the pancreas, isolated pancreatic localization has not been reported previously. METHODS A 68-year-old white female presented with a multicystic mass in the pancreas. Fine needle aspiration showed some protoscolices, free hooklets and fragments of laminated layer, which are pathognomonic features of echinococcosis. Serologic analyses by an enzyme-linked immunosorbent assay using Em2plus antigen showed high antibody reactivity of the patient's serum, which is indicative of an infection with E multilocularis. Diagnosis was finally confirmed by molecular and immunologic analyses of the cytologic material by polymerase chain reaction and direct immunofluorescence. CONCLUSIONS This case illustrates the value of cytology in the identification of echinococcosis, particularly when it involves extrahepatic sites, where the risk of misdiagnosis may be related to its extremely rare occurrence. Precise parasitologic tying of E multilocularis in endemic areas is important.