Hereditary angioedema is a rare and life threatening autosomal-dominant disorder which results from the congenital deficiency of C1-esterase inhibitor. It is responsible for approximately 2% of all angioedema cases. Recurring angioedema attacks that involve subcutaneous and submucosal areas are the hallmarks of hereditary angioedema. Here, we review the clinical findings and therapeutic approaches of the disease by presenting a 5-years old female patient with severe extremity edema who was diagnosed as hereditary angioedema and treated with fresh frozen plasma.