Subretinal neovascularization associated with retinochoroidal coloboma is rare. To the authors' knowledge, only three cases have been reported. We report a new case of isolated subretinal neovascularization and coloboma in a 26-year-old woman without other causes of choroidal neovascularization. Only the disruption of the normal retinal anatomy could explain the development of this subretinal neovascularization.