Retinoblastoma is a malignant intraocular tumor of childhood that requires accurate diagnosis and prompt treatment. It is well known that several other ocular conditions of childhood can clinically simulate retinoblastoma. Knowledge of the clinical features that serve to differentiate retinoblastoma from simulating lesions may assist the clinician in arriving at the correct diagnosis and preventing misdirected therapy. This review provides a simple classification of those pseudoretinoblastomas and reviews the recent literature on this subject. It emphasizes the clinical features that help differentiate pseudoretinoblastomas from true retinoblastoma.