This case report describes an infra-diaphragmatic sequestrated type II congenital cystic adenomatoid malformation (CCAM) containing striated muscle fibers in the stroma occurring in association with an extra-lobar sequestration (ELS) and a congenital diaphragmatic hernia in a female neonate. The classification and pathogenesis of CCAM and ELS are reviewed and the relationships between these lesions discussed.