Since its initial recognition, approximately 100 cases have been reported. Yet, the true incidence is unknown, because many patients are asymptomatic. The diagnosis is usually made in the second or third decade of life, and recognition is often incidental. Although bronchial atresia has been reported in stillborn babies and in a sexagenarian, the average age at diagnosis is 22 years. A male predominance of the disorder is noted. While most individuals remain asymptomatic, others require evaluation for recurrent infections, unexplained dyspnea, and pulmonary nodules, or abnormal radiograph findings. Symptomatic patients tend to present at earlier ages, whereas older patients tend to be more asymptomatic. In patients with bronchial atresia, the physical examination findings are usually unremarkable, but can reveal focal diminished breath sounds, wheezing, and/or tachypnea.