Intrapulmonary sequestrations are quite uncommon in pediatric age group. The preoperative diagnosis of pulmonary sequestration is not possible in most of the cases. A 2-year-old boy presented with recurrent episodes of chest infections and respiratory distress. A preoperative diagnosis of congenital lobar emphysema was made on the basis of chest radiograph and computed tomography scan. At operation, an intralobar pulmonary sequestration was found. The sequestration cyst was excised with uneventful recovery.