Intestinal malrotation includes anomalies of intestinal rotation and fixation occurred during embryogenesis. It may present with acute or chronic-intermittent symptoms or remain asymptomatic. There are several methods to identify a defect of intestinal rotation, however an upper gastrointestinal series remains the method of choice in non-acute cases. Surgical correction is performed by open laparotomy, while the laparoscopic approach is reserved only to selected cases. This article suggests an evaluation of children with suspected intestinal malrotation and gives information about its management and complications to paediatricians.