The congenital pulmonary airway malformations (CPAM) are extremely rare malformations which are most frequently diagnosed before birth or during childhood and are extremely rarely diagnosed in adults. Sometimes, type II CPAM can be associated with pulmonary sequestrations (PS), especially those extralobar. We present the case of a 39 years old female without any known previous respiratory symptoms until this age, who has having for approximatively 2 months repeated episodes of haemoptysis. The angio-CT exam showed the existence of multiple cyst-like formations in the left inferior pulmonary lobe (LLL) and 2 arteries starting from thoracic aorta that participated at the vascularization of the LLL. We performed a left inferior lobectomy and the postoperative evolution was favorable. The histopathological exam confirmed the diagnosis of type I CPAM associated with intralobar PS. The particularity of the presented case is given by the fact that this patient of 39 years old had no previous respiratory symptoms and no cases of type I CPAM associated with intralobar PS were cited before.