Abstract
Congenital absence of the portal vein (CAPV) is a rare defect often accompanied by other abnormalities such as heart defect, skeletal anomalies and / or liver tumors [1]. We describe here a case of CAPV revealed by an upper gastrointestinal tract hemorrhage in a child aged 02 years and 8 months. The esogatro-duodenal fibroscopy revealed the presence of Esophageal varices grade II, abdominal ultrasound revealed a large liver with no visualization of the spleen. Subsequent abdominal computed tomography revealed the presence of a spleno-mesaraic trunk measuring 7 mm in anteroposterior diameter receiving the inferior and superior mesenteric vein and a small splenic vein draining by dilated peri-gastric and peri-esophageal leads. Associated with splenic hypoplasia and portal trunk atresia. The rest of the malformative assessment was negative
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