Paediatric retinal disease is the most important part of paediatric ophthalmology. It usually manifests as leukocoria or yellow pupil, typically in retinopathy of prematurity, familial exudative vitreoretinopathy, persistent hyperplastic primary vitreous, Coats disease and retinoblastoma. It also can be manifested as nystamus poor visual fixation or progressive worsening of visual function, typically in Leber congenital amaurosis, Stargardt disease, Best disease and cone and rod dystrophy. Paediatric retinal diseases can be roughly divided into acquired, hereditary and congenital developmental abnormalities. With the development of gene and stem cell technologies, the advent of new medicine, equipments and new techniques, the concept of diagnosis and treatment in paediatric retinal diseases is also changing. In China, the level is improving progressively in both clinical and research areas of paediatric retinal diseases.