Abstract: Objectives　To investigate the clinical manifestation and distribution characteristics of aortopulmonary collateral arteries (APCAs) without cyanotic congenital heart defect, and to analyze the reason of misdiagnosis. Methods　The clinical data of 137 APCAs patients without cyanotic congenital heart defect in our hospital from April, 2009 to October, 2010 were reviewed. Clinical characteristics, radiographic findings were analyzed. Results　In 137 patients, 99 were combined with pulmonary diseases, 115 cases with heart diseases. Six cases were misdiagnosed in 137 patients. Three cases were misdiagnosed as patent ductus arteriosus. One case was misdiagnosed as cardiomyopathy. One case was misdiagnosed as pulmonary tuberculosis. One case was prolonged the time of antituberculosis therapy because of the lung lesion. There were 130 APCAs (94.9%) originated from descending aorta, five (3.6%)  from subclavian artery, one (0.73%) from intercostals artery, one (0.73%）
 from vertebral artery. Conclusions　APCAs exist in the patients without cyanotic congenital heart defect, and APCAs will be narrowed and blocked gradually after birth. However, certain predisposing factors sustain APCAs and increase the pulmonary flow. Patients with APCAs combined with pulmonary disease or heart diseases may be misdiagnosed or diagnosed as a specific disease. Early diagnosis and treatment is critical to prognosis.