Gastro-intestinal stromal tumours are uncommon malignancies of the gastro-intestinal tract, accounting for only 0.2% of all gastro-intestinal malignancies, but are the most common of abdominal sarcomas. Classically, they have been considered amenable only to early stage surgical intervention. Recent advances in targeted cancer therapies have led to the development of effective non-surgical treatment options. This article discusses the epidemiology and physiopathology of, as well as treatment options available for, this uncommon disease.