Optical coherence tomography has improved the ophthalmic clinical examination due to its ability to more accurately characterize pathology of the eye. Optical coherence tomography is now an integral imaging modality for many adult diseases of the retina and is a ubiquitous part of a retinal practice. However, although its use in adults is well recognized, the adoption of optical coherence tomography in pediatric patients may be undervalued as macular diseases in children are relatively uncommon. Recent technological advancements have made optical coherence tomography scans quicker and more precise, generating normative data and leading to more widespread use in children. In this review article, we discuss how pediatric optical coherence tomography data can be used to understand normal development, as well as diagnose and monitor retinal disease. In particular, we highlight clinical optical coherence tomography findings of several pediatric retinal conditions, including retinopathy of prematurity, congenital infections, retinal dystrophies, and phakomatoses such as neurofibromatosis.