Intralobar pulmonary sequestration (ILPS) is a rare congenital malformation of the lung. It is described as a segment of lung parenchyma with normal pleura and systemic blood supply and it has poor communication with the tracheobronchial tree. Patients usually present in later childhood or adulthood with a history of recurrent pneumonias. The malformation is rarely associated with other congenital anomalies such as a congenital pulmonary airway malformation (CPAM). A CPAM is a congenital cystic lesion of the lung that presents usually in the newborn period as respiratory distress. We describe the case of a 2-month-old female who presented to the local emergency room with the initial diagnosis of pneumonia, surgical diagnosis of ILPS, and a final pathological diagnosis of ILPS with an associated CPAM.