Pulmonary sequestration and congenital broncho-esophageal fistula are both rare malformations; nonetheless, in the presence of recurrent pulmonary infection, the possibility that one or the other may be involved should not be disregarded. To our knowledge, the concurrence of intralobar pulmonary sequestration and congenital broncho-esophageal fistula is very rare. Herein, we report a case of intralobar pulmonary sequestration combined with congenital broncho-esophageal fistula, both of which were successfully treated with surgery.