In the past, patients underwent pulmonary plombage to render the empty hemithorax. Plombage, also known as extraperiosteal or extrapleural pneumonolysis, is a historical treatment procedure for cavity tuberculosis of upper lobes of lungs used between the 1930s and 1950s. During the operation, a cavity is created by the intrathoracic placement of inert materials, commonly Lucite (acrylic) balls, ping pong balls, oils, rubber sheets, paraffin wax, and gauze. As a result, the mediastinal and skeletal shift toward the volume loss side. Recent approach involves the implantation of tissue expander either via open thoracotomy or thoracoscopically in an attempt to shift the mediastinum back to its anatomical location. Such surgical procedure involving the implantation of tissue expander for treating pulmonary agenesis was first reported in Berlin, Germany which was then followed by reports from Bordeaux, France and Verona, Italy. The main focus of the treatment procedure is to preserve the remaining functional tissue and prevent significant musculoskeletal disfigurement that may arise as a side effect of tissue expander implantation, as the mediastinal and skeletal shift towards the volume-loss side. If the defect is extensive but there is a chance for the fetus to live, an exo-utero intrapartum treatment (EXIT) may be performed to salvage the potential life. EXIT technique involves partial delivery of a baby through an incision in the uterus while remaining attached to their mother's placenta. Such procedure is necessary for babies who require airway support so that they are provided with a functioning airway before they are detached from their mother's placenta. The EXIT procedure is used to perform lung resection for babies with extensive lung defects in a relatively stabilized condition after birth. Prognosis of pulmonary agenesis depends on the degree of pulmonary involvement during the embryonic stage of lung development, as well as the patient's history of pulmonary infections and the presence of associated anomalies. The majority of patients diagnosed with bilateral pulmonary agenesis die in utero or within the first few hours after birth. Numerous cases of bilateral pulmonary agenesis, where both lungs have been affected, have been reported previously. On the other hand, the hypertrophy of the remaining lung to compensate for the lost lung is common in the case of unilateral pulmonary agenesis. However, the mortality rate still exceeds 50%.