Purpose: To report a case of juxtapapillary polypoidal choroidal neovascularization (PCNV) associated with choroidal nevus investigated by means of optical coherence tomography angiography (OCT-A). Methods: Case report. Results: A 72-year-old woman presented with visual loss and metamorphopsia in her left eye for 5 days secondary to PCNV that developed on the border of a juxtapapillary choroidal nevus. Fluorescein angiography, indocyanine green angiography, and spectral-domain OCT confirmed the diagnosis. En face OCT-A disclosed a large tangled hyperreflective PCNV spreading from the optic disc at different levels of the choriocapillaris; the polyp lumina appeared hyporeflective. The patient was treated with 3 intravitreal injections of anti-vascular endothelial growth factor with partial functional recovery. Conclusions: Our case showed the application of OCT-A in the diagnosis of a case of active PCNV complicating a benign intraocular tumor. © 2016 Wichtig Publishing.