The case of a 50-year-old woman with an extremely rare venous malforma
tion of the portal venous system is reported. The patient presented wi
th a true aneurysm of the superior mesenteric vein, which has thus far
been reported in no more than eight cases worldwide. this malformatio
n may be congenital or acquired. Secondary aneurysms are thought to be
due to liver disease, portal hypertension, trauma, or inflammation. A
neurysms of the portomesenteric venous system may be asymptomatic or g
ive rise to severe, often dramatic conditions such as crampy abdominal
pain, jaundice, and upper gastrointestinal hemorrhage secondary to po
rtal hypertension. The diagnosis is usually made by ultrasound (B-mode
or color flow Doppler), CT scan, and MRI. Invasive procedures such as
venous phase mesenteric arteriography or splenoportography may be hel
pful in confirming it. in our opinion aneurysms of the portal venous s
ystem, even if they are congenital and (still) asymptomatic, require e
arly surgical control because the prognosis for patients with these an
eurysms is unpredictable and potential complications (e.g., portal hyp
ertension, fistula, contained perforation, or rupture) may be fatal. I
n the case presented the mesenteric venous aneurysm was resected and t
he confluent veins were reconstructed.