Background: Congenital pulmonary airway malformation (CPAM) is a rare congenital abnormality with unknown exact etiology or clear genetic association. It is characterized by failure of bronchial development and localized glandular over growth. Schizencephaly is another condition described as a rare congenital disorder of neuronal migration characterized by developmental malformation of the cerebral cortex characterized by dysmorphic gray matter lined clefts in the cerebral cortex filled with cerebrospinal fluid, extending medially from the subarachnoid space into and continuous with the ipsilateral lateral ventricle. Case Report: We present a rare case of a young lady presenting with an acute chest infection, found to have asymmetry in her body development, the right half of her body was underdeveloped with growth retardation, eventually diagnosed to have both schizencephaly and congenital pulmonary airway malformation. Conclusion: Future detection and description of such cases including presentation and natural history is of pivotal importance since case prevalence is exceedingly rare.