Extralobar sequestration is a congenital acquired disease more frequently observed after birth or during infancy. In half of the cases, it is associated with another malformation. In the following case we report the observation of a 24-year-old female with right extralobar sequestration associated with a diaphragmatic hernia and containing gastric mucosa and a congenital cystic adenomatoid lung malformation. Such malformative lung tissue is known to potentially degenerate and justifies surgery even when patients are asymptomatic.
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