Objective: Sinistral, or left-sided, portal hypertension (SPH) is a rare cause of upper gastrointestinal hemorrhage. This retrospective analysis summarizes the clinical features of SPH and the results of surgical treatment. Subjects and Methods: Between 2000 and 2009, patients from our hospital diagnosed with SPH were identified. Diagnosis of SPH was based on evidence of isolated splenic vein thrombosis, splenomegaly, gastroesophageal varices and exclusion of other causes of portal hypertension. Results: Eight males and 5 females were included in the study, with a mean age of 43.5 ± 6.4 years (range: 31'68 years). Liver function was normal in all patients. Causes of SPH were chronic pancreatitis (n = 7), pancreatic cancer (n = 3), pancreatic cysts (n = 2) and neuroendocrine tumor (n = 1). The main clinical manifestations were gastrointestinal hemorrhage in 7 cases (53.8%), upper abdominal pain in 10 (76.9%) and hypersplenism in 12 (92.3%). All patients had splenomegaly and gastroesophageal varices. Twelve patients underwent splenectomy and 1 received surgical removal of a pancreatic cyst. No major gastrointestinal tract rebleed occurred after a mean follow-up of 46 months (±7 months). Two patients died of pancreatic cancer and 1 of acute myocardial infarction during follow-up. Conclusions: SPH should be suspected in patients with upper gastrointestinal varices as well as unexplained splenomegaly with normal liver function. Surgical intervention such as splenectomy offers a good long-term outcome in symptomatic patients.