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With the improved survival rate of patients with retinoblastoma, the incidence of second malignancies has become substantial. We had two retinoblastoma survivors with secondary malignancies in the naso-ethmoidal complex, one a 21-year-old man with anaplastic carcinoma and the other a 16-year-old girl with malignant fibrous histiocytoma. They underwent repeated craniofacial surgery. Secondary malignancies arising at the nose and ethmoidal cells often invade the anterior skull base. The introduction of skull base surgery has provided wider surgical margins and increased the resultant likelihood of cure. Both patients have so far shown no evidence of recurrence.