• We reviewed the records of 50 patients with heritable retinoblastoma to study the cause of death, if deceased, or the incidence of second nonocular malignant neoplasms. The incidence of second tumors in these patients was 6% at ten years, 14% at 20 years, and 14% at 30 years. These findings are lower than previously reported results. Pinealoma was the probable cause of death in three of the five patients who died of second nonocular tumors.