Retinoblastoma is the most common intraocular tumor of childhood. Although retinoblastoma classically presents with leukocoria and strabismus, the definitive diagnosis is made by evaluating fundoscopic findings together with the findings of ancillary tests such as ultrasonography, fundus fluorescein angiography, optical coherence tomography and magnetic resonance imaging. The management of the disease is closely related to the presence of sporadic or hereditary disease, the presence of unilateral or bilateral disease and the presence of local or distant metastases. The aim of this article is to summarize the findings of the traditional methods together with the innovations in the diagnostic tools of retinoblastoma, and to explain the classification systems and the treatment methods adopted accordingly.