We have estimated the incidence of retinoblastoma in the United States from data available form the Surveillance, Epidemiology, and End Results program of the National Cancer Institute, Bethesda, Md (1974 through 1985). The larger number of cases available (220) compared with those of previous US population-based studies enabled us to evaluate change in incidence over time and to more precisely estimate incidence according to various demographic characteristics. The incidence of retinoblastoma has been almost uniform form 1974 to 1985. The average annual incidence of retinoblastoma was 5.8 per million for children younger than 10 years and 10.9 per million for children younger than 5 years. There was no difference in the incidence of retinoblastoma by either sex or race. The overall 5-year cumulative survival rate was 91% (95% confidence interval, 87% to 95%). The data indicate a worsening survival with increasing age at diagnosis, through age 2 years, but a less clear relationship of survival with diagnosis beyond age 2 years.