We report a case of a patient known to have a von Hippel-Lindau disease with documented progressive juxtapapillary retinal capillary hemangioma (JRCH) with well-preserved visual acuity (VA) and visual field (VF). The patient received a single injection of intravitreal ranibizumab (IVR). Six months after IVR injection, the JRCH showed reduced vascularization, fibrosis, and mild shrinkage, and VA and VF remained unchanged. IVR therapy might therefore be considered as an alternative treatment for progressive JRCH, especially in patients with well-preserved VA and VF.