Hereditary angioedema (HAE) is a rare autosomal dominant condition characterized by episodes of swollen of the upper airway, intestines, and skin. The primary pathogenesis is the reduction in the function and/or plasma concentration of complement 1 esterase inhibitor (C1-INH). When edema involves the gastrointestinal tract, abdominal pain will be the main manifestation, which is often difficult to differentiate from surgical acute abdomen and may lead to misdiagnose and mistreatment. This paper reported a case of HAE with recurrent abdominal pain as the main manifestation, which diagnostic course was rugged and complicated. Eventually, the diagnosis was confirmed by testing the function and concentration of C1-INH, and a satisfactory clinical outcome was achieved after prophylactic treatment with Lanndelumab.

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