Intestinal malrotation with volvulus is generally presented as a bilious vomiting and acute intestinal obstruction in the newborn period. It could compromise vascular supply of the small bowel secondary to torsion of superior mesenteric artery (SMA) and without urgent surgical management, it could lead to detrimental outcomes such as transmural bowel infarction and sepsis. However, in chronic cases, it is rarely obstructs the vascular supply and propagates to an acute bowel infarction. Rarely, chronic malrotation with midgut volvulus may not reduce the mesenteric blood supply because of collateral vessels, and the chronically stagnated blood flow of the superior mesenteric vein (SMV) favors thrombus formation within the lumen. The recommended treatment is Ladd's procedure and anticoagulation therapy. The authors present an unusual case of intestinal malrotation with chronic volvulus resulting in superior mesenteric vein and portal vein thrombosis in a 28-year-old patient.