Hereditary angioedema (HAE) is an infrequent disorder characterised by abnormalities in the levels and/or function of complement C1 esterase inhibitor (C1 INH). Clinical manifestations of HAE are due to recurrent episodic swelling of the subcutaneous or submucosal tissue. When swelling involves the gastrointestinal mucosa, patients may present with nausea, vomiting, diarrhoea, and severe abdominal pain. However, HAE is almost never suspected as a potential cause of acute abdomen. We describe a typical case of hereditary C1 esterase inhibitor deficiency in a 37-year-old woman presenting with recurrent acute abdomen and who had undergone negative surgical interventions before diagnosis was eventually established. Both the diagnostic and therapeutic approach to this rare condition is discussed in this report.