Nephrotic syndrome (NS) is a protein-losing enteropathy that can present as a surgical emergency or complicate routine surgical procedures. This case highlighted the hypercoagulable state that resulted in significant small bowel ischaemia and necrosis. Mesenteric ischaemia as a complication of NS has not been reported in the paediatric population. The postoperative overwhelming sepsis also highlighted the effect of impaired immunity in NS on patient outcomes.