Pleuropulmonary blastoma is an extremely rare lung neoplasm exclusive to children under 5 years of age. It presents a diagnostic challenge both prenatally and in early childhood due to its similarity to benign lung cysts, which are managed differently. We present the first case, to our knowledge, of a neonate with pleuropulmonary blastoma and myelomeningocele, though prenatally diagnosed as a congenital pulmonary airway malformation. We detail the prenatal imaging that facilitated counseling and delivery management in addition to the correlating postnatal imaging.