Portal-systemic shunt, spontaneous connection between systemic veins and portal vein system, is a rare cause among the patients with hepatic encephalopathy. We reported a case of 57-year-old male with extrahepatic portal-systemic shunt who presented with repeated episodes of disturbance of consciousness and characteristic images of abdominal sonography, contrast-enhanced abdominal computed tomography and 3D reconstruction images. There are a few reports in the literature about symptomatic portal-systemic shunt presented in adulthood and it may be misdiagnosed as psychological diseases. So the diagnosis of this rare disease may rely on abdominal image studies only and associated symptoms may be resolved by avoidance of precipitating factors, surgical occlusion or radiological obliteration.