Pulmonary sequestration is a rare and complex congenital malformation. In most cases, the abnormality is detected in early childhood and is to be surgically corrected; however, there are cases of diagnosing the defect in older and even senile ages. The concurrence of intrapulmonary sequestration and lung cancer is even less common; the world literature currently describes isolated cases of detection of two abnormalities in one patient. It depicts a 64-year-old patient who saw a physician for recurrent right lower lobe pneumonia. The examination of the patient revealed the concurrence of intralobar sequestration and squamous cell carcinoma of the right lower lobe. The patient underwent surgical treatment as expended right lower bilobectomy with ligation of the aberrant artery of the lower lobe of the lung.