The aim of this study was to establish the causes of leukocoria in pediatric patients in a tertiary hospital environment. All patients younger than 10 years who presented with leukocoria between January 1, 1999, and December 31, 2000, were included in the study; 39 (55%) patients were boys and 32 (45%) patients were girls. Sixty percent of the patients who presented with leukocoria had congenital cataract (18% unilateral and 42% bilateral). Other causes included retinoblastoma (11% unilateral and 7% bilateral), retinal detachment (2.8% unilateral and 1.4% bilateral), bilateral persistent hyperplastic primary vitreous (4.2%), and unilateral Coats' disease (4.2%). Leukocoria in children demands immediate attention because a significant number of children have pathology that either threatens life or causes permanent visual disability.