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PURPOSE To describe the role of optical coherence tomography angiography (OCTA) to help in differential diagnosis between papilledema and a rare case of choroidal neovascularization (CNV) associated with Morning Glory Syndrome (MGS). METHODS Observational case report. RESULTS A 22-year old man was referred to Eye clinic from Neurological unit with a diagnosis of papilledema in right eye. OCTA scans on the optic disc revealed a dense radial peripapillary vascular network while two polypoidal choroidal vasculopathy (PCV) were present in peripapillary region at the edge of staphyloma in corrispondence of choriocapillary layer. Therefore, the patient underwent three-monthly intravitreal injections of anti-vascular endothelial growth factor. One and six months after loading phase, the PCVs showed no clinical activity at structural spectral domain-OCT and OCTA. CONCLUSION This case revealed the crucial role of OCTA as an efficace diagnostic technique in discriminating papilledema from MGS complicated by CNV, allowing to make differential diagnosis, useful in ophthalmological and neurological clinical practice.