Recurrence of retinoblastoma usually occurs within the first few years after treatment. Despite complete tumor regression, delayed relapse of retinoblastoma can occur, predisposing patients to osteosarcoma as second malignancy. Our case was unique regarding the late relapse 13 years after treatment and recurrence at the femur. The initial tumor was treated with chemotherapy, enucleation of the right eye, and plaque radiotherapy. The relapsed tumor was treated with chemotherapy, consolidation, and autologous stem cell transplant. Our patient is currently in complete remission and continues to follow-up with oncology and ophthalmology. This report illustrates the possibility of late recurrence of retinoblastoma.