INTRODUCTION:Hereditary angioedema is an inborn error of immunity, due to complement deficiency, with regular prevalence in relation to other disorders of the immune system. There are three types of angioedema with pictures of subcutaneous and mucosal edema that can aggravate with airway compromise. In Ecuador it remains a rare disease, with little diagnostic approach. CASE PRESENTATION: A 17-year-old patient with recurrent facial edema from an early age, managed as allergic processes, born to non-consanguineous parents, deceased mother with a history of crises, without timely therapeutic approach, siblings with similar edema, as well as grandmother and maternal uncle. She was diagnosed as hereditary angioedema by the immunology service, going to the emergency room with facial edema, right forearm and a sensation of suffocation and at 9 weeks of gestation, it was decided to transfer her to intensive care for management and control of the feasibility of the product. Due to a lack of human C1 esterase inhibitor, fresh frozen plasma was administered, doses of tranexamic acid, which began to improve her general situation, edema completely decreased 2 days after hospitalization. She presented low C4 (value of 3), the only test carried out due to lack of resources, a sample is extracted for a molecular study, and she is kept in periodic controls by the Immunology and Gynecology service, already completing her 24 weeks of gestation with a single-sex product male. DISCUSSION: Hereditary angioedema, a disease little known in Ecuador by the health system in general, and more so in obstetrics and gynecology. Lack of resources delays approach and treatment, being the clinical history, signs and symptoms pathognomonic of the disease. It is necessary to establish a clinical care protocol, especially in pregnant women, which would help the quality of care for these patients, with a probable decrease in morbidity and mortality