Primary intestinal lymphangiectasia (PIL) is a rare disorder in children and it is more exceptional in adults. PIL is caused by a diffuse or localized dilatation and/or rupture of intestinal lymphatic vessels in the mucosa, submucosa, or subserosa due to high pressure in lymphatic vessels. The diagnosis is made on clinical grounds with the support of small bowel biopsies. The following report present a case of intestinal lymphangiectasia revealed by capsule endoscopy examination. This work shows that standard EGD and colonoscopy may miss characteristic lesions of PIL, and capsule endoscopy (or enteroscopy) may be required for the diagnosis because lesions are typically located in distal duodenum/jejunoileum.