Hereditary angioedema is a rare disorder characterized byrecurrent angioedema attacks due to C1 inhibitor antigen orfunctional deficiency. Here, two cases with recurrent swelling onextremities, genital organs and face that were later diagnosedwith C1 inhibitor functional deficiency (hereditary angioedematype 2) were presented. The first patient was an 8-year-oldboy complaining of hand and foot swelling recurring once ayear for the last 4 years. He was more frequently brought tooutpatient pediatric clinics because of his recurring complaintsin the last 5 months. In prodromal period, he had erythemamarginatum-like rash and sometimes described abdominalpain with swelling. The second patient was an 11-year-old girlcomplaining of abdominal pain and facial swelling recurring ina couple of months for the last 8 years. Her grandfather, fatherand uncle had the same angioedema. In their laboratoryevaluation, complement C4 levels were both found as low at<0.07 g/L (>0.1). Although C1 inhibitor antigen levels wereboth in reference range, its function tests were detected as lowat 31% and 43% (>70%), respectively. Therefore, in cases withpediatric recurrent angioedema without urticaria, hereditaryangioedema should be considered. After screened with C4level, if required, both C1 inhibitor antigen and function testsare evaluated.