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Background: Children diagnosed with the hereditary form of retinoblastoma (Rb) have excellent survival, but face an increased risk of bone and soft tissue sarcomas. This predisposition to sarcomas has been attributed to genetic susceptibility due to inactivation of the RB1 gene as well as past radiotherapy for Rb.
Objectives: To report the case of a child with hereditary form of bilateral RB, who developed Ewing’s sarcoma of the right femur 10 years after the Enucleation of the both eyes.
Methods: The child was diagnosed as a case of bilateral RB at the age of 3 months. He was fully investigated and found to have locally advanced RB. Enucleation was done. The patient received chemotherapy and radiotherapy. At 14 years, he was investigated for a small swelling in his right lower leg. After extensive investigations, it was reported as Ewing’s sarcoma. He is treated with chemotherapy.