We present a case of intestinal malrotation with an absent inferior vena cava, which was found in a cadaver during a dissection course in our medical school. The intestinal malrotation was Amir-Jahed type 2, with the large intestine on the right side and the small intestine on the left side of the abdominal cavity. The descending colon was fixed on the right side of the posterior abdominal wall and continued into the pelvic cavity from the right side. The cadaver also had a venous system anomaly. The pre-renal segment of the inferior vena cava, which is a section between the renal vein and the hepatic vein, was absent. The inferior vena cava connected to the azygos vein after being joined by bilateral renal veins. The only hepatic segment of the inferior vena cava, which was posterior to the liver and received hepatic veins, penetrated the diaphragm and flowed into the right atrium. To our knowledge, this is the first report of these two anomalies appearing concurrently. We discuss the details of this case and the embryological considerations.
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