A pancreatic-portal vein fistula (PPVF) is an extremely rare complication of pancreatitis. In this report, we present a patient with a PPVF in the setting of recurrent pancreatitis and portal vein thrombosis diagnosed by cross-sectional imaging and endoscopic retrograde cholangio-pancreatography, who responded to medical management and anticoagulation. A critical review of this disease process is explored to highlight pathology, imaging characteristics, and essential alternative diagnostic considerations. We also discuss potential complications and current treatment strategies. PPVFs pose a unique diagnostic challenge and awareness of its clinical presentation can further improve patient outcomes.
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