A juxtapapillary retinal capillary hemangioma (JRCH) is a rare vascular hamartoma located on the optic nerve head or adjacent region. While often associated with von Hippel-Lindau (VHL) disease, JRCHs can also occur as an isolated condition, presenting unique therapeutic challenges and risks of visual impairment. We report a case of a 50-year-old Malay gentleman with diabetes mellitus who presented with a non-progressive superior visual field defect in his left eye for three months. Fundus examination revealed a raised reddish mass adjacent to the nasal optic disc, accompanied by macular exudates. Optical coherence tomography confirmed cystoid macular oedema, while fundus fluorescence angiography revealed feeder and draining vessels with late leakage and adjacent small vessel vasculitis. Systemic evaluation found no evidence of VHL or other abnormalities. Despite treatment with photodynamic therapy and intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections, the patient's vision progressively deteriorated. This case highlights the diagnostic and therapeutic challenges associated with JRCH and emphasizes the need for early recognition and tailored interventions to mitigate significant visual impairment.