Intralobar sequestration (ILS) is an uncommon abnormality that accounts for 75％ of all pulmonary sequestrations.Over the years there have been several reports of various presenting signs of which hemoptysis was commonly described,however,massive hemoptysis and hemothorax is extremely rare in literature.We present a case of a 45-year-old man who died of fatal complication from an ILS.This case report shows an uncommon presentation of ILS with massive hemoptysis and hemothorax resulting in a dramatic course of disease and a fatal outcome,and for this reason in the absence of trauma or other causes for massive hemoptysis,hemothorax,or lung hematoma,this possibility should be kept in mind so as to avoid misdiagnosis,and resection of the sequestered tissue should be considered in all patients.