Of 23 cases of metastatic retinoblastoma treated between 1922 and 1979, seven had metastases limited to the cranial vault and 13 had cranial metastases plus distant metastases. Globe pathology showed invasion of the optic nerve and/or the choroid in all but two patients, but was not predictive of the metastatic pattern. Initial signs of metastases were neurologic impairment and an orbital or body mass; first symptoms were anorexia or weight loss, vomiting, and headache. Most cases occurred by 3 years of age. Death occurred within 5.8 months on the average, despite therapy. Useful tests for determining the extent of disease were bone marrow aspiration, lumbar puncture, skull films, EEG, and brain scan. Computed tomographic scans of the head, bone scans, bone marrow aspiration, automated blood chemistry analysis, and lumbar puncture with immediate ethyl alcohol processing should prove to be useful to detect metastatic disease.