Inflammatory bowel disease (IBD) is a chronic inflammatory disorder of intestines associated with a large number of extraintestinal manifestations (EIMs) which significantly contribute to its morbidity and mortality. A 32-year-old man presented with ulcerative colitis (UC) accompanied by the unusual coexisting clinical manifestations of primary sclerosing cholangitis, thromboembolism, and hemolytic anemia during the disease course. The incidental finding of hemochromatosis of the liver was attributed to chronic hemolysis. The full-blown EIMs and systemic complications in this patient did not appear to be attributable to drug treatment and/or infection but might have been related to both the underlying immune-mediated mechanism and the lack of early and adequate treatment for UC and its associated complications. This case emphasizes the need for early and aggressive treatment of IBD accompanied by EIMs to avoid morbidity and mortality.