Hereditary angioedema (HAE) is a rare inherited disorder characterized by increased vascular permeability. Most patients present with decreased levels and/or impaired function of C1 esterase inhibitor (C1-INH). However, a small proportion of patients have normal C1-INH levels and function, which makes the diagnosis more challenging.Here, we report a case of ANGPT1 mutation-associated HAE (HAE-ANGPT1) presenting with recurrent abdominal pain as the initial manifestation. The patient experienced repeated episodes of abdominal pain, while complement C4 levels as well as C1-INH concentration and function were within normal ranges. During attacks, abdominal imaging revealed intestinal wall edema and thickening, whereas gastrointestinal endoscopy showed no definite organic lesions. Whole-exome sequencing identified a mutation in the angiopoietin-1 gene (ANGPT1), leading to the diagnosis of HAE-ANGPT1.After treatment with lanadelumab, the frequency and severity of abdominal pain attacks were significantly reduced, although occasional recurrences still occurred. During follow-up, the patient achieved good symptom control after avoiding potential triggers, including psychological stress, infection, and mechanical trauma.This case suggests that HAE should be considered in patients with recurrent abdominal pain characterized by intestinal wall edema on imaging but negative endoscopic findings. For patients with normal C1-INH levels and function, genetic testing is helpful for establishing a definitive diagnosis.