An ectopic varix of the small intestine is a very rare vascular abnormality, characterised by obscure gastrointestinal bleeding outside of common locations. Idiopathic ectopic varices are cases without known risk factors, such as portal hypertension, venous thrombosis or previous surgery. Only a few cases have ever been reported in the literature. In this study, we present two cases of idiopathic jejunoileal varices with microscopic evidence, with similar cases reviewed from the literature. The aetiology, diagnosis and treatment of idiopathic small intestinal varices are also reviewed in this report. A pan-endoscopy and colonoscopy usually reveal negative results in such patients, and angiography and contrast abdominal computed tomography are often used for timely diagnosis. Surgical intervention is mostly performed, as it is an efficient treatment method with a good prognosis.