Mesenteric cystic lymphangiomas are uncommon benign cystic tumors rarely associated with other congenital malformations. We herein describe a case of a 9-day-old female presented with a two-day history of bilious vomiting, feeding intolerance, weakness and lethargy. Preoperative studies were diagnostic of intestinal malrotation. At laparotomy a cystic mesenteric mass was identified. Histopathological examination confirmed the diagnosis of mesenteric cystic lymphangioma. The exact aetiology of mesenteric lymphangioma is unknown and this case supports the theory that lymphangioma is a primary congenital malformation.