Retinoblastoma is a malignant tumor that arises from the retina. The prognosis is very unfavorable if the choroid is affected or the central nervous system is invaded or if metastasis have occurred. We experienced a case of the retinoblastoma with optic nerve involvement in a 3-years-old female had metastasis to the tibia. She was enucleated of the left eye five months ago. We report it with brief review of the literature.