Ulcerative colitis is a common chronic inflammatory disease of the colon and rectum, resulting from a dysregulated immune response towards intraluminal antigens in a genetically predisposed host. The disease has a varying extent and severity. Approximately 20% of patients with ulcerative colitis experience a severe flare during the course of their disease, requiring hospitalization. Acute severe ulcerative colitis (ASUC) is potentially a life-threatening condition that requires early recognition, hospitalization, correction of body fluids and electrolytes, and nutritional support if needed. Superimposed bacterial or viral infections need to be excluded and thromboprophylaxis should be started. Intravenous corticosteroids are the first-line treatment for this condition. Rescue treatment with ciclosporin or infliximab is indicated in patients who do not sufficiently respond to corticosteroids after 3-5 days, with close monitoring of the patients' symptoms, serum C-reactive protein and albumin levels. If medical therapy fails, timely colectomy should be performed to prevent critical complications. In this article, we review all relevant aspects of ASUC, from its pathophysiological background to modern management in clinical practice.