Splanchnic arteriovenous malformation (AVM) is a rare condition in which patients present with portal hypertension, which thus causes bleeding varices and ascites. However, to our knowledge, hepatic nodules associated with splanchnic AVM have not yet been described. We herein first report the case of a 78-year-old man with inferior mesenteric AVM presenting with portal hypertension and multiple hepatic nodules dominantly supplied by the portal vein. This unique case not only extends the spectrum of hepatic nodules resulting from abnormal hepatic circulation, but also provides clues for better understanding the etiology of hepatic nodules.