Retinoblastoma is a malignancy of the human developing retina. In situ as well as in vitro studies have attributed tumoral histogenesis either to a primitive retinoblast with neuronal and glial differentiation potentials, or to a photosensory progenitor cell. Here it is shown in vivo that the retinal topography of 457 retinoblastoma and retinoma foci is radially asymmetrical. Tumor density appears to mimic the horizontal visual streak characteristic of red/green cone cell distribution. Such a non-random distribution seems to invalidate the hypothesis of a primitive multipotential neuroblast as the unique source of retinoblastoma and may support the view that retinoblastoma evolves along the cone cell lineage.