Juxtapapillary capillary hemangiomas can occur as isolated clinical abnormalities or a manifestation of von Hippel-Lindau (VHL) disease. Because of the location of these vascular tumors on or adjacent to the optic disc, any therapeutic treatment option involves risk. Management typically includes close monitoring, with treatment initiation as the tumor progresses or when vision is compromised. This case report reviews this condition while highlighting the treatment and potential complications associated with juxtapapillary capillary hemangioma. A 14-year-old black male had juxtapapillary capillary hemangioma diagnosed during a routine eye examination. The patient complained of blurry vision in the right eye, and best-corrected visual acuities (BCVA) were 20/25 O.D. and 20/20 in the left eye. At the retinologist's discretion, no treatment was initiated, and the patient was advised to return in 4 months for a dilated retinal evaluation and referred for magnetic resonance imaging (MRI) with contrast of the brain. The patient was lost to follow-up and presented a year later when vision had deteriorated to 20/400 O.D. with an associated afferent pupillary defect. Enlargement of the juxtapapillary capillary hemangioma with increased fibrovascular growth, macular exudation, and subretinal fluid was observed O.D. Photodynamic therapy was initiated, and the patient experienced treatment complications, including a vitreous hemorrhage and serous retinal detachment. A scleral buckling procedure, vitreoretinal surgery, and endo-laser photocoagulation were performed. The patient's visual acuity improved from 20/400 to 20/60 O.D., with the development of a dense posterior subcapsular cataract, for which the patient had cataract extraction. Genetic testing was positive for a small deletion in codon 76 of the von Hippel-Lindau gene, and MRI of the brain was reported to be normal. Additional testing to rule out visceral organ involvement, including abdominal ultrasound scan, urinalysis, and blood chemistry were ordered, and the patient's family was also evaluated for the presence of the VHL gene. The clinical course of juxtapapillary capillary hemangiomas is highly variable, potentially leading to significant ocular complications. Currently, no definitive treatment guideline is available, and any therapeutic option involves inherent risk because of the location of these tumors. Because juxtapapillary capillary hemangioma may be the only manifestation of VHL disease, genetic and clinical screening of patients and their relatives is highly recommended. Early diagnosis and treatment may improve both the visual and systemic prognosis in patients with juxtapapillary capillary hemangiomas.