Hereditary Angioedema (HAE) is a rare disorder caused by C1 esterase inhibitor deficiency or dysfunction. Patients with HAE usually present without urticaria or pruritis affecting the skin, upper airway, or the gastrointestinal tract. They can also present with involvement of unusual sites making the diagnosis challenging and leading to unnecessary testing and complications. Prompt diagnosis and treatment is crucial to prevent mortality and morbidity associated with acute flare. Here we present, what is believed to be second case of isolated involvement of the jejunum from an attack of HAE.