Retinoblastoma is the most frequently diagnosed intraocular malignancy in infants. Recently, there have been significant advances in the management of this disease. New therapeutic strategies, such as superselective intra-arterial chemotherapy and periocular/intravitreal chemotherapy, have been added to conservative treatments with a long consolidated efficacy. All these procedures are available at the Referral Centre for Retinoblastoma of the University Hospital of Siena. These advances have been reflected in increased rates of eye salvage and improved visual outcomes, notwithstanding the primary objective to safeguard the lives of affected children. This article summarizes the most important clinical and therapeutic aspects of this malignancy that every paediatrician should be aware of, particularly when retinoblastoma is suspected in a young patient.