A 33-year-old woman with a history of hereditary bilateral retinoblastoma as an infant presented to a primary care clinic with 1 week of right lower extremity swelling. At age 8 months, she was treated with right enucleation and left eye radiotherapy (50.4 Gy). At age 4 years, she developed a recurrence of the retinoblastoma in the nasal cavity and was treated with 4 cycles of vincristine, doxorubicin, and cyclophosphamide followed by 1 cycle of cisplatin, etoposide, and intrathecal cytarabine.