Pulmonary sequestration (PS) and congenital cystic adenomatous malformation (CCAM) are both rare congenial disorders with similar etiologies. PS derives a vascular supply from systemic circulation, but does not connect to the tracheobronchial tree. CCAM is characterized by the formation of a cyst with a pulmonary vascular supply that connects to the tracheobronchial tree. Intrapulmonary PS rarely occurs with CCAM. We describe the case of a 63-year-old woman who had intrapulmonary PS, with pathology results indicating a coexistence with CCAM.