In the pathology of respiratory tract, sequestration presents as a non-functional lung tissue with no communication with tracheobronchial tree. It represents a rare congenital bronchopulmonary and vascular malformation, which occurs at a frequency of 0.1 to 6%. Intralobar and extralobar sequestrations are extremely rare congenital anomalies, especially if they occur at the same time in a patient. Proper diagnosis and appropriate surgical therapy, in the absence of associated anomalies, provide an excellent prognosis. In this paper, we are describing the simultaneous presence of intralobar sequestration (ILS) in the lower lobe of the left lung and extralobar sequestration (ELS) which is positioned on the aortic arch, in a 53 years old man. Two years post surgery, there is no recurrence or any patomorphological and functional disorders in the respiratory tract.