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Objectives: Congenital diaphragmatic hernia (CDH) has been associated with other congenital malformations. Intestinal rotation abnormalities are relatively common in CDH, due to abnormal rotation of the midgut. The incidence of malrotation in CDH patients has been previously described. However, there is no standard evaluation and treatment of malrotation during initial CDH repair. This study evaluates risk factors for co-existence of an intestinal malrotation in CDH patients, its initial treatment and whether they can cause any further abdominal problems. Methods: All patients with a CDH treated in a high volume center between 2000 and 2015 were retrospectively evaluated. Demographics, CDH specific characteristics, surgical treatment, and short/long term abdominal outcomes were described. Specific outcomes were malrotation at initial CDH repair, malrotation during follow up and occurrence of small bowel obstruction. All (surviving) patients had a minimum follow up of 18 months. Differences were calculated using the independent student's T-test or Mann-Whitney U test for continuous variables and chi-square or Fisher exact tests for categorical data, as appropriate. Results: In total 215 patients were included, of which 197 were surgically repaired. In 76 (39%) a malrotation was described at initial CDH repair, in 39 (20%) a normal rotation, but in 82 patients (42%) there was no report on intestinal rotation. There were no significant differences between these groups regarding demographics. During follow-up (range 1.5-16 years) twelve additional malrotations were diagnosed, leading to 45% (n=88) prevalence. These were diagnosed due to acute small bowel obstruction, of which three had a volvulus. More than half of these missed malrotations (58%) required acute surgery for treatment of malrotation, compared to only two of the initial 76 patients (p<0.001). Patient with no report on rotation of the intestines were most likely to develop small bowel obstruction and require surgery during follow up (table 1). A subanalysis showed that in this patient cohort, 174 patients had Bochdalek hernia, 13 patients an eventeration, 7 patients a Morgagni hernia, two patients had pentalogy of Cantrell and one unknown. A malrotation was eventually evaluated in 45% of Bochdalek hernia patients, 23% of the eventeration patients and 86% of Morgagni patients (p=0.03). Conclusion: Intestinal malrotation is associated with congenital diaphragmatic hernia, with a prevalence of at least 45%. It is clear that a missed malrotation has a very high risk on small bowel obstructions and acquiring acute laparotomy. Therefore, it is extremely important to diagnose and treat malrotation in CDH patients to prevent acute small bowel obstruction, including volvulus. [ABSTRACT FROM AUTHOR]