Congenital absence of the splenic artery is a very rare condition.To the best of our knowledge, congenital absence of the splenic artery accompanied with absence of the splenic vein has not been reported.We report a case of the absence of the splenic artery and vein in a 61-year-old woman who presented with postprandial epigastric discomfort. Upper gastrointestinal endoscopy showed a dilated, pulsatile vessel in the fundus and duodenal stenosis. An abdominal computed tomography(CT)scan revealed absence of the splenic vein with a tortuously engorged gastroepiploic vein.Three-dimensional CT demonstrated the tortuously dilated left gastric artery and the left gastroepiploic artery with non-visualization of the splenic artery.After administration of a proton pump inhibitor,abdominal symptoms resolved without any recurrence of symptoms during 6 mo of follow-up. Congenital absence of the splenic artery is a very rare condition. To the best of our knowledge, congenital absence of the splenic artery accompanied with absence of the splenic vein has not been reported. We report a case of the absence of the splenic artery and vein in a 61-year-old woman who presented with postprandial epigastric discomfort. Upper gastrointestinal endoscopy showed a dilated, pulsatile vessel in the fundus and duodenal stenosis. An abdominal computed tomography (CT) scan revealed absence of the splenic vein with a tortuously engorged gastroepiploic vein.Three-dimensional CT demonstrated the tortuously dilated left gastric artery and the left gastroepiploic artery with non-visualization of the splenic artery. After administration of a proton pump inhibitor, abdominal symptoms resolved without any recurrence of symptoms during 6 mo of follow-up .