Malignant complication of pulmonary bullous disease is rare but not negligible. Its importance lies in the young age when it appears. Eighty-nine cases in the literature, including four of our own cases, are included in this review. Etiopathogenic theories are discussed along with clinical, radiological, pathological, diagnostic, prognostic, and treatment characteristics. Smoking is a primary factor in this process, and quitting is key in terms of prevention. Surgical methods are usually necessary to perform the diagnosis, but that does not exclude the use of other techniques-particularly, fine-needle aspiration biopsy under radiological control-whose role should be validated. The prognosis is bad if there is no commitment to prevention. Even though the value of serial radiological controls is unknown, such controls may help in the early detection of neoplastic degeneration in bullous disease. The nature of the problem in handling these cases therapeutically is determined by the severity of bullous dystrophy: therefore, surgery should be considered on an individual basis.