This chapter provides dedicated discussion on the intracranial disorders of neuro-ophthalmologic consequence in children. An integrated approach to these diseases has emerged from the proliferation of multidisciplinary clinics and programs combining expertise in pediatric neurology, neurosurgery, neuropathology, neuroradiology, neuro-oncology, and neuro-ophthalmology. The molecular and genetic aberrations involved in the pathogenesis of many of these disorders have only recently been elucidated. Refinement in neurosurgical management continues to advance the treatment of these disorders, while preventative and therapeutic measures will arise from molecular genetic research. Advances in neuroimaging have revolutionized the diagnosis of intracranial disease in children, while recent documentation of increased cancer risk in children exposed to multiple computed tomographic (CT) scans has emphasized the need to order diagnostic magnetic resonance (MR) imaging when neuroimaging is warranted .