Pulmonary sequestration is a rare congenital lung malformation characterized by an abnormal segment of bronchopulmonary tissue supplied by aberrant systemic arteries. Due to the non-specific symptomatology, the diagnosis can be missed. Imaging is the cornerstone of the diagnosis. Complete surgical resection provides the definitive treatment. We report a case of pulmonary sequestration associated with an asymptomatic aspergillosis presenting during adulthood and describe briefly the epidemiology, embryology, histology, imaging and surgical treatment of this congenital abnormality.