Intestinal lymphangiectasia(IL) is an uncommon protein losing enteropathy, characterized by small intestinal mucosa or serosa lymphangiectasia and intestine lymph loss. Currently, IL is a very rare disease in children or adults, with typical clinical symptoms including hypoalbuminemia, absolute lymphocyte reduction, ascites, edema, etc. We report a case of an adult with intestinal lymphatic ectasia accompanied by chylothorax and multiply arteriovenous malformations of the hip and lower extremity. CT and MRI revealed diffuse edema and thickening of the small intestine, accompanied by splenomegaly and pleural effusion. Extensive nodularity of lower ileum and the ileocecal region could be seen during intestinal endoscopy. Finally, small intestinal lamina propria lymphangiectasis was confirmed by pathological examination. To raise awareness of the disease, here we compare our case and those previously reported, and discuss the diagnosis and management of IL. Intestinal lymphangiectasia (IL) is an uncommon protein losing enteropathy, characterized by small intestinal mucosa or serosa lymphangiectasia and intestine lymph loss. Currently, IL is a very rare disease in children or adults, with typical clinical symptoms including hypoalbuminemia, absolute lymphocyte reduction, ascites , edema, etc. We report a case of an adult with intestinal lymphatic ectasia accompanied by chylothorax and multiply arteriovenous malformations of the hip and lower extremity. CT and MRI revealed diffuse edema and thickening of the small intestine, accompanied by splenomegaly and pleural effusion. Extensive nodularity of lower ileum and the ileocecal region could be seen during intestinal endoscopy. Finally, small intestinal lamina propria lymphangiectasis was was by pathological examination. To raise awareness of the disease, here we compare our case and those previously reported, and discuss the diagnosis and management of IL.