Background Portal vein aneurysms are rare dilations in the portal venous system, for which the etiology and pathophysiological consequences are poorly understood. Method We reviewed the existing literature as well as present a unique anecdotal case of a patient presenting with a very large portal vein aneurysm that was successfully managed conservatively and non-operatively without anticoagulation, with close follow-up and routine surveillance. Result The rising prevalence of abdominal imaging in clinical practice has increased rates of portal vein aneurysm detection. While asymptomatic aneurysms less than 3 cm can be clinically observed, surgical intervention may be necessary in large asymptomatic aneurysms (>3 cm) with or without thrombus, or small aneurysms with evidence of evolving mural thrombus formation on imaging. Conclusion Portal vein aneurysms present a diagnostic challenge for any surgeon, and the goal for surgical therapy is based on repairing the portal vein aneurysm, and if portal hypertension is present decompressing via surgically constructed shunts.
