Background: To describe the clinical features and course of a series of survivors of retinoblastoma who developed a non-pineoblastoma second primary malignant neoplasm (SPMN). Methods: A retrospective review of patients treated for retinoblastoma who developed a SPMN was performed. The demographics, clinical features and treatments for retinoblastoma, pathologic types of SPMN, the intervals between the retinoblastoma diagnosis and treatment and diagnosis of non-pineoblastoma SPMN, treatment provided for the SPMN, and the survival outcomes of the patients were evaluated. Results: Of 550 patients treated initially for retinoblastoma, this series used the 15 (2.7%) that developed a non-pineoblastoma SPMN, 14 of which (93.3%) had been treated for bilateral retinoblastoma. All patients in this series had germline retinoblastoma. The median time from retinoblastoma diagnosis to SPMN diagnosis was 19.0 years (extremes 3.4 and 39.4 years). Six of the fifteen patients died during the follow-up of their SPMN. Nine patients were still alive without active residual SPMN at the last follow-up. The median interval between initial retinoblastoma diagnosis and death in the 6 patients who died of their SPMN was 18.8 years (extremes 6.2 and 34.6 years) and between diagnosis of the SPMN and death was 1.2 years (extremes 0.25 and 4 years). Conclusion: A SPMN occurred in this series in 2.7% of retinoblastoma survivors, and all occurred in patients with germline retinoblastoma. All patients with SPMN who had been treated by EBRT developed the SPMN in the field of prior radiation.