Hereditary angioedema (HAE) is an important disease characterized by recurrent episodes of angioedema without urticaria, which most often affects the skin or mucosal tissues of the upper respiratory, genitourinary and gastrointestinal tracts. The angioedema is mostly self-limited and resolves in a few days without treatment, however in some conditions such as upper respiratory involvement, may cause fatal asphyxiation. Gastrointestinal attacks are experienced by a majority of patients with HAE and can be the principal presentation. The diagnosis of hereditary angioedema may be delayed in many cases. Many patients with undiagnosed HAE may undergo inappropriate abdominal surgery. In this study, we are presenting a 14 years-old patient and his mother who are diagnosed with type 1 HAE, predominantly suffering from abdominal pain attacks due to intestinal angioedema. The patient underwent appendectomy and two times diagnostic laparotomy at different times due to unexplained abdominal pain. With this case report we wanted to raise awareness of physicians about HAE.