The aim of this article is to present a clinical picture of congenital optic nerve anomalies, which occur quite frequently in the general population, may cause low vision and visual field defects. These abnormalities can coexist with central nervous system defects or the other ocular abnormalities. Some of them may be misdiagnosed as papilloedema during the episode of intracranial fluid pressure elevation or glaucomatous neuropathy. Diagnosing the congenital nerve optic disc defects coexisting with multiorgans anomalies requires multidisciplinary medical care especially in babies. developmental optic disc anomalies, congenital eye abnormalities, differential diagnosis.