Enfisema lobar congénito en los lóbulos superior y medio del pulmón derecho en una paciente de 2 meses de edad

background. bronchopulmonary malformations demonstrate a low frequency in the population. their presentation is sporadic and may be associated with malformations in other systems. within this group is congenital lobar emphysema, which is a developmental anomaly of the lower respiratory tract manifesting as hyperinflation of one or more lung lobes. among its causes, entrapment of air through a valve mechanism at the bronchus or gigantism by overdevelopment of lobular alveolar lung parenchyma has been proposed. case report. we report the case of a 2-month-old female patient who from birth presented with respiratory disease classified as pneumonic process. the patient was treated with multiple antibiotic schemes as well as placement of pleural seals complicated by pneumothorax and pleural effusion. subsequently, diagnosis of congenital lobar emphysema was made and supported by laboratory studies. surgery was carried out where involvement of the middle and upper lobes of the right lung was demonstrated. conclusions. the patient presented a favorable evolution with a good expansion of residual right lung and correction of mediastinal positions.