Background: Clinical diagnosis of malrotation is difficult to achieve, especially in older children and adults as its nonspecific presentations at this stage are usually neglected leading to delayed diagnosis of these cases. Objectives: In this study, we aimed to evaluate the diagnostic values of ultrasound and upper gastrointestinal (GI) series in patients with suspected intestinal malrotation. Methods: This six-year cross-sectional, prospective study was conducted in Dr. Sheikh Children's Hospital affiliated to Mashhad University of Medical Sciences, Mashhad, Iran from 2009 to 2015. Totally, 67 children (aged < 15 years) with clinical findings of intestinal malrotation who had evidence of malrotation in ultrasound or upper GI series were enrolled in the study. Results of radiological evaluation were compared to the final diagnosis of patients achieved from surgery. Data analysis was performed using SPSS software. Results: Of the 67 enrolled participants with clinical findings or suspected radiological signs of intestinal malrotation, about half (52.2%) were male. Patients were 2 days to 16 years (mean, 2.5 years) old. Associated anomalies were observed in 47.7% of cases. Sensitivity and specificity were 82.5% and 85.7%, respectively for GI series findings and 82.3% and 54.5%, respectively for ultrasound compared with surgical findings as a gold standard. Conclusions: Ultrasound has a similar diagnostic value compared to upper GI series. They are complementary examinations and negative ultrasound or GI series results do not necessarily rule out intestinal malrotation. Mesenteric vessels ultrasound could be used as an accurate diagnostic method for the detection of intestinal rotational disorders.