Drug-induced liver injury (DILI) is a spectrum of pathology that can be classified by mechanism of injury or by type of observed hepatotoxicity. Vanishing bile duct syndrome (VBDS) is a group of acquired and genetic disorders that cause the destruction and disappearance of intrahepatic bile ducts, and cholestasis. VBDS typically presents with severe cholestatic hepatitis and can have immunoallergic features. Infliximab has been reported to rarely cause a cholestatic pattern of liver injury due to ductopenia characteristic of VBDS. Herein we present a clinical case of infliximab-induced DILI resulting in VBDS.