Retinoblastoma is the most common intraocular malignancy in infancy and early childhood, posing a serious risk to vision and life. It has a familial tendency and occurs mostly under 3 years of age, often in one eye or both eyes. According to the general development of retinoblastoma, it can be clinically divided into 4 stages, namely, intraocular growth phase, intraocular pressure increase phase (glaucoma phase), extraocular extension phase and systemic metastasis phase. Intraocular growth phase: The early signs and symptoms are visual impairment and fundus changes. Due to the loss of vision, the pupil opens wide and a yellowish-white reflection is visible through the pupil, which is called “cataract”. Cataracts are the most easily detected early clinical symptom of the disease. In fact, when the yellowish-white reflection appears in the pupil, the disease has already progressed to a significant degree. Intraocular tumor growth can lead to increased intraocular pressure, causing obvious headache, eye pain, conjunctival congestion, corneal edema and other glaucoma symptoms. Extraocular extension stage: The earliest occurrence is the spread of tumor cells along the optic nerve into the skull, and the tumor penetrates the sclera into the orbit, resulting in protrusion of the eye; it may also cause corneal chylomicron or penetrate the cornea and grow outside the ball, or even protrude beyond the lid fissure and grow into a huge tumor. Systemic metastasis stage: late stage tumor cells may metastasize to the skull via optic nerve; metastasize to lymph nodes and soft tissues via lymphatic vessels; or metastasize to bones, liver, spleen, kidney and other tissues and organs via blood circulation. It eventually leads to death. Commonly used treatment methods include surgery (including eye removal, orbital content removal), external radiation therapy, local treatment (photocoagulation, cryotherapy, heating therapy, superficial scleral patch radiation therapy) and chemotherapy. In the past 10 years, international treatment has been dominated by the gradual rise of chemotherapy combined with multiple aggressive local treatments as the first-line treatment, the reduction of external radiation therapy to the second-line treatment, and the removal of the eye as the third-line treatment. This change is considered to have ushered in a new era in the treatment of retinoblastoma.