We describe congenital colobomatous malformations of the optic nerve and the clinical variations and complications that may appear eventually in them. We also present two clinical cases of patients with papillary colobomatous defects where spontaneous variations have been observed throughout their evolution and where the current ophthalmoscopic aspect is completely different from the initial one. Finally, bearing in mind the embryologic origin and histologic structure of these anomalies, we develop a hypothesis that explains the physiopathologic mechanism that causes the clinical changes described in each case.