Leukocoria is a whitish pupillary reflex resulting from retrolental abnormality. Leukocoria presents in childhood, and is an important alerting sign of intraocular pathology. In particular, leukocoria is the most common presenting sign of retinoblastoma. Neuroradiological examinations may help in the differential diagnosis between retinoblastoma and simulating lesions, such as persistent hyperplastic primary vitreous, Coat's disease, retinopathy of prematurity, etc. Close interdisciplinary collaboration between neuroradiologist and ophthalmologist is essential to provide accurate early diagnosis and proper treatment.