Retinocytoma is a benign variant of retinoblastoma that closely resembles a focal regressed or treated retinoblastoma. It was initially thought to have resulted from spontaneous regression of retinoblastoma. However, histopathologic studies showed these tumors are composed of well-differentiated, benign-appearing mature photoreceptor cells without characteristics of malignant transformation or necrosis to suggest regression. Retinocytoma is a benign manifestation of the RB1 gene. Translucent grey appearance, dull and chalky calcification, retinal pigment epithelial alternations, and a zone of surrounding chorioretinal atrophy are characteristic features of retinocytoma. Retinocytoma is not associated with prominent feeder vessels or retinal exudation. Retinocytoma is nonprogressive but does rarely undergo malignant transformation. Retinocytoma carries similar genetic implications as germinal retinoblastoma.