Congenital cataracts are cataracts that are present before and after birth, with a small percentage forming soon after birth. The main risk is to cause blindness or amblyopia in infants and children. Cataracts block normal light from entering the eye and stimulating the retina, resulting in abnormal retinal development. Therefore, early diagnosis and treatment of congenital cataract is the key to preventing amblyopia in children with cataract, and delayed treatment will lead to lifelong amblyopia and disability in children with cataract. Congenital cataracts can be classified as familial onset or as disseminated cases. About 1/3 of patients have genetic factors, and consanguineous marriage is a risk factor. Non-hereditary cataracts are associated with intrauterine viral infection in the fetus during pregnancy (rubella infection in the second trimester), malnutrition, metabolic disorders, oxygen absorption after birth, and exposure to radiation. Congenital cataracts need to be differentiated from retinoblastoma, advanced retinopathy of prematurity, endophthalmitis, permanent primitive vitreous hyperplasia (PHPV), severe retinal developmental abnormalities, and Coats disease. Treatment of congenital cataracts consists of two major components: surgical removal of the cataract and refractive reconstruction. While the surgical part is relatively simple, refractive reconstruction is a very daunting task for these children, especially in monocular cataracts. This includes the choice of IOL prescription and long-term postoperative amblyopia training. This is mainly due to the fact that normal young children have a great ability to adjust the lens, while surgically implanted IOLs do not have this ability to adapt to the growth of the eye. The earlier the congenital cataract surgery is performed, the better. This is determined by the child’s general condition and degree of clouding. The treatment of congenital cataract is not only surgery, but more importantly, long-term post-operative amblyopia training and follow-up (usually until the age of 10). Congenital cataracts should be followed up regularly for at least 18 months after surgery. The general chance of posterior cataract in children is close to 100%. If it affects the central light-transmitting area it should be operated on secondarily. Otherwise the previous treatment is meaningless.