Abstract: Objective To improve the recognition and diagnosis and treatment of congenital absence of left pulmonary artery accompanying pulmonary hypertension. Methods Two cases of congenital absence of left pulmonary artery accompanying pulmonary hypertension were reported and related literature was reviewed. Results The two male patients were admitted to our hospital due to paroxysmal chest distress and short breath because of exertion,and diagnosed as congenital absence of left pulmonary artery accompanying pulmonary hypertension by right heart catheterization and pulmonary arteriography.Their condition was relieved after treatment with oxygen inhalation,cardiac tonics,diuretics,and pulmonary hypertension-reducing drugs. Conclusion Since the incidence of congenital absence of left pulmonary artery is low and lacks of specificity,it is easily misdiagnosed and even its diagnosis is missed.Its early diagnosis and treatment can improve the quality of life of such patients and prolong their life.