Pulmonary sequestration (PS) is a rare congenital lung malformation presented as non-functioning lung mass, abnormally supplied by an aberrant systemic artery. The diagnosis of PS requires a high index of clinical suspicion. Patients may be asymptomatic or have wide range of clinical manifestations including recurrent pneumonia, hemoptysis, chest pain and respiratory distress. The gold standard diagnostic tool of PS is CT angiography (CTA). The treatment includes open surgical resection, video-assisted thoracoscopic surgery (VATS), and endovascular embolization. This mini-review highlights the literature for the contemporary methods of diagnosis and treatment of PS.