This report describes a case of adult-onset cystic fibrosis (CF) in an African-American male. Although CF is a common autosomal recessive disorder in populations of European descent, it is relatively rare in the African-American population (1 in 17,000), with only Asian population ancestries being less affected than African blacks. We present our patient's disease course in order to elucidate the manifestations of CF in this particular ethnic population. More specifically, this patient had a form of CF with late-onset features, which may represent a new clinical phenotype of CF. We seek to improve clinician awareness of CF disease subtypes. We also show the radiographic intra- and extra-thoracic manifestations of CF and the peculiar clinical scenario that brought forth this condition.