Localised dilatation of a segment of the intestine without any macroscopically-identifiable cause is rare, and has been reported in association with omphalocoele in only 14 children up to 2006. In most of these cases, the segmental intestinal dilatation (SID) was either diagnosed incidentally, or due to presentation with partial or complete intestinal obstruction. We report, for the first time, a 37-week-old neonate with bowel perforation in SID associated with omphalocoele. In our case, a long thin vessel that resembled the mesodiverticular vessel of a Meckel's diverticulum was present in the dilated segment, supporting the view that SID and Meckel's diverticulum may be embryologically related.