Segmentary idiopathic splenic vein stenosis is a very rare condition. We report a unique case of acute gastric variceal bleeding in a 31-year-old pregnant woman with left-sided portal hypertension from segmentary idiopathic splenic vein stenosis. Hemorrhage was controlled by endoscopic acrylate glue injection and urgent cesarean section allowed successful delivery. The patient declined subsequent intervention and has been on beta-blockers with no bleeding recurrence since then. This condition, its pathophysiological implications and management are discussed.
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