Radiologists must be able to recognize the imaging signs of intestinal malrotation because this condition can lead to potentially lethal complications such as midgut volvulus. The correct diagnosis depends on both high clinical suspicion and the radiologist's ability to recognize the specific signs of malrotation and the normal variants that can lead to the wrong diagnosis. Although the location of the third portion of the duodenum outside the retroperitoneal area on ultrasonography, CT, or MRI seems to be a reliable sign of malrotation, the gold standard for determining whether the duodenojejunal flexure is in an abnormal location continues to be the upper gastrointestinal series. In this article, we review the most important imaging signs of malrotation and emphasize the role of ultrasonography in diagnosing midgut volvulus.