Left-sided portal hypertension is a rare clinical syndrome which may lead to bleeding from isolated gastric varices. Pancreatic disease is the most common etiology. Left-sided portal hypertension should be considered in the presence of gastrointestinal bleeding with normal liver function and unexplained splenomegaly. It may be difficult to diagnose this entity both endoscopically and radiologically. While splenectomy is the treatment of choice for cases complicated by variceal bleeding, there is no consensus on the treatment of asymptomatic patients. The prognosis of left-sided portal hypertension mainly depends on the underlying etiology.