Metastatic retinoblastoma is uncommon in high income countries. It occurs in less than 5% of cases of retinoblastoma, usually as a relapse few months following enucleation of intraocular disease, especially in cases with high-risk pathology features. Metastatic retinoblastoma is not curable with conventional treatment. Metastatic retinoblastoma is the most common cause of death from this tumor worldwide. Late diagnosis and poor compliance to recommended treatment are the most important factors associated with the occurrence of metastasis in lower income countries. Treatment with high dose chemotherapy and autologous stem cell rescue is the only curative treatment currently available.