The most frequent primary intraocular malignancies are uveal melanoma in adults and retinoblastoma in children. Genetic findings in uveal melanoma now allow a better risk-prediction with regard to metastatic disease. New treatment modalities like endo-resection, trans-scleral resection, proton beam irradiation and trans-pupillary thermotherapy are now being established in clinical routine. Management of retinoblastoma has changed during the last years considerably. In hereditary retinoblastoma external beam radiotherapy (EBR) results in a sixfold increased risk for the development of secondary, non ocular malignant tumors in these patients. New treatment regimens based on systemic chemotherapy were developed to replace EBR. In combination with chemotherapy there has been a continuing trend toward more conservative focal treatment for retinoblastoma. Indications and first results of these new treatment modalities are presented.