Objective To investigate the application of RetCam Ⅱ examination in the screening of infantile eye diseases. Methods The clinical data of infants aged less that 1 year undergoing fundus examinations by RetCam Ⅱ between January 2012 and June 2012 were collected, and the birth history (birth weight and gestational age), adjusted gestational age, gender and family history of diseases were recorded. Results A total of 903 infants (1 806 eyes) received RetCam Ⅱ examination, and there were abnormal findings in 890 eyes (49.28%). There were 50 eyes with abnormal anterior segment, 784 eyes with abnormal posterior segment, 26 diseased eyes combined with systematic disease, 26 eyes with phthisis bulbi and 4 eyes with trauma. Among the 784 eyes with abnormal posterior segment, there were 416 eyes (53.06%) with retinopathy of prematurity, 132 eyes (16.84%) with familial exudative vitreoretinopathy and 90 eyes (11.48%) with persistent fetal vasculature. Among the 50 eyes with abnormal anterior segment, those with congenital cataract accounted for 56.00% (28 eyes). Conclusion RetCam Ⅱ plays an important role in the early screening of infantile eye diseases, especially for congenital and hereditary fundus diseases.