We report a case of a 2 years and 8 months boy presented with a small bowel obstruction occurring in a setting of mesenteric lymphatic malformation (LM). The case did not showed typical features of LM and thought to be a simple ascites. Nevertheless, the mass effect of the lesion was the salient finding and the case presented with a small bowel internal hernia which is a rare presentation. This emergent case was managed surgically.