Ocular manifestations that occur directly or indirectly as result of a pathologic process that involves other parts of the body, in a pediatric population, will be discussed here. While a myriad number of systemic conditions have ocular manifestations, its importance cannot be undermined because it has implications for both diagnosis and treatment. Often, the eye findings can give a clue to the systemic diagnosis and at other times, not managing the eye manifestations can lead to irreversible blindness although the systemic condition was treated well. So, it is important for all clinicians dealing with pediatric population to be aware of ocular manifestations of the common systemic conditions. The authors discuss the ocular manifestations of the following systemic conditions: Genetic and chromosomal anomalies, phakomatoses, metabolic disorders, infectious diseases, craniofacial anomalies, muscular disorders, inflammatory disorders and miscellaneous.