Intestinal malrotation is a developmental anomaly resulting from embryologic failure of fixation and rotation of the gut and predisposed to midgut vovulus and small bowel obstruction. Although various forms of malrotation anomalies in the gut can occur, nonrotation of prearterial segment with the initial symptomatic presentation at advanced age has been rarely reported. Here, we report a case of nonrotation of the prearterial segment of the midgut presenting with duodenal obstruction in a 60-year-old man. To our best knowledge, this is the first case reported about nonrotation of prearterial segment of the midgut in Korea.