Vascular anomalies of the large bowel, commonly known as vascular malformations of the colon (VMC), constitute a rare but important condition, potentially causing significant morbidity and mortality. Our aim is to provide an up-to-date, practical summary evaluating this disease entity, focussing on pathogenesis, as well as diagnostic and therapeutic modalities. We reviewed available data in the literature, and discussed it in the form of a narrative, readily applicable review. Most VMC (over 70%) are detected in the caecum and ascending colon, and affect people aged over 50 years. VMC are almost always symptomatic, presenting with lower bleeding. Endoscopy is crucial to identify and locate VMC, and to treat the lesions. In patients who fail or do not fit endoscopic treatment, aggressive approaches (interventional angiology or surgery) are mandatory. Up to 40% of patients may have relapse in the long term. VMC are rare but potentially life-threatening. Advances in endoscopic imaging and therapy have improved the results of treatment. Long-term follow-up after treatment is recommended.