Abstract: Objective：To investigate the incidence of congenital heart disease in patients with congenital ptosis and the relationship between the two diseases. Methods：The incidence and types of congenital heart disease were retrospectively analyzed in 1 053 patients with congenital ptosis. Results：Forty patients were found to have different types of congenital heart diseases among all of these 1 053 patients with congenital ptosis, yielded an incidence of 3.80%, which was higher than that in the general population. Of the 1 053 patients with congenital ptosis, 174 cases, 416 cases, and 463 cases were diagnosed as mild, moderate, and severe ptosis respectively. There were 2 cases (1.15%), 11 cases (2.64%), and 27 cases (5.83%) with congenital heart disease in children with mild, moderate and severe ptosis respectively. Patients with severe ptosis had a significantly higher incidence of congenital heart disease than those with mild or moderate ptosis (P<0.05). And 664 cases and 389 cases had unilateral and bilateral ptosis respectively. There were 11 cases (1.66%), 29 cases (7.46%) with congenital heart disease in patients with unilateral and bilateral ptosis respectively. Children with bilateral ptosis had a significantly higher incidence of congenital heart disease than those with unilateral ptosis (P<0.01). Conclusions：The incidence of congenital heart disease is increased in patients with congenital ptosis. Higher risk of congenital heart disease may exist in patients with severe and bilateral ptosis. Screening for congenital heart disease in children with ptosis is necessary.