Abstract
BACKGROUND: Congenital unilateral lung agenesis is a rare condition in which there is complete absence of one lung, in addition to the associated bronchus and pulmonary vasculature. It can be an isolated finding or present in conjunction with cardiac, gastrointestinal, genitourinary and skeletal malformations. Due to the increasing physiological demands on the body during gestation and parturition, this condition poses significant risks in individuals with this condition. CASE: A 26-year-old G1P0 with known right lung agenesis was managed with a multidisciplinary approach by obstetrics, maternal-foetal medicine, pulmonology and cardiology specialists. The patient subsequently had an uncomplicated pregnancy and vaginal delivery. CONCLUSION: Multidisciplinary care led to an optimal pregnancy outcome in a woman with congenital unilateral lung agenesis. Women with this condition with normal baseline function have a good prognosis in pregnancy.