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In a series of 882 retinoblastoma patients, 384 known to have the genetic form of the disease and 498 others, 30 patients developed second primary neoplasms. The spectrum of these second neoplasms is discussed in relation to the forms of treatment used for the retinoblastoma. Cumulative incidence rates of second tumours in the whole series are 2.0% at 12 years after diagnosis and 4.2% after 18 years. For patients with the genetic form of retinoblastoma the cumulative incidence rate after 18 years is 8.4% for all second neoplasms and 6.0% for osteosarcomas alone. The inherent risk among survivors from genetic retinoblastoma of developing an osteosarcoma, excluding all possible effects of treatment, is estimated to be 2.2% after 18 years. Within the field of radiation treatment the cumulative incidence rate for all second neoplasms after 18 years is 6.6% and for osteosarcomas alone 3.7%. There is some evidence that patients with genetic retinoblastoma are particularly sensitive to the carcinogenic effects of radiation. The results also suggest that the use of cyclophosphamide may increase the risk of second primary neoplasms in patients with genetic retinoblastoma. The incidence rates of second primary neoplasms in retinoblastoma survivors reported here are lower than those quoted for previously published series. Evidence from this and other papers strongly suggests an association between retinoblastoma and malignant melanoma. Retinoblastoma is a rare childhood tumour which can occur either unilaterally or bilaterally, and which may be either familial or sporadic. Cases known to be familial, and those with bilateral disease, usually show a pattern of inheritance consistent with the view that the occurrence of the tumour is attributable to a dominant autosomal gene with a penetrance of around 90%. We shall refer to familial and bilateral cases as 'genetic'. Patients who survive genetic retinoblastoma have a substantial risk of developing osteosarcoma, probably soft tissue sarcomas and possibly other types of neoplasm. Reese et al. (1949) reported two second tumours following treatment of 55 cases of bilateral retinoblastoma with combined radiation and surgery, and the high risk of second malignant neoplasms in retinoblastoma survivors has subsequently been well documented These second primary neoplasms can often be attributed to radiotherapy but many cases have been reported of second tumours occurring outside the field of radiation treatment (Aherne, 1974; Abramson et al., 1976), and, in some instances, where no radiotherapy had been given (Abramson et al., 1979). It has been suggested that patients …