Bilateral intralobar and extralobar pulmonary sequestration is an extremely rare anomaly. At present, four pathologically proven cases and two possible cases have been reported in the literature insofar as we know. We have found no previous reports in the literature in which simultaneous bilateral intralobar and extralobar pulmonary sequestration were present. We report a first case presenting as bilateral intralobar and extralobar pulmonary sequestration, recently performed staged bilateral resection, and emphasize the importance of computed tomography and angiography in diagnosis and operation.