Abstract Symptomatic, as well as asymptomatic, splenic vein thrombosis in patients with pancreatic inflammatory disease, appears to be more common than is generally recognized. The diagnosis should be suspected in every patient who presents with variceal bleeding in the absence of signs of liver disease. Preoperatively, the diagnosis can be readily established by angiography. During an emergency operation for gastrointestinal bleeding, the finding of a normal liver, an enlarged spleen, and a markedly dilated gastroepiploic vein should alert suspicion of this condition. The diagnosis is confirmed if upon catheterization of the portal vein both the pressure and the venographic appearance of that vessel are shown to be normal. The syndrome is one of the rare instances where the problem of portal hypertension is cured by splenectomy. In the presence of splenic vein thrombosis, cystojejunostomy is perferable to cystogastrostomy for the treatment of a pancreatic pseudocyst, because the latter procedure carries a risk of bleeding from the transected varices in the posterior wall of the stomach. Should emergency splenectomy be needed, only external drainage of an associated pancreatic pseudocyst may be feasible.