Objectives:The aim of the study was to evaluate the clinical and optical coherence tomography (OCT) findings of patients with choroidal neovascularization (CNV) due to best vitelliform macular dystrophy (BVMD).Methods:Six eyes that were diagnosed with CNV associated with BVMD were evaluated retrospectively. A standard ophthalmologic examination, a fundus fluorescein angiography (FA), and the OCT findings of the patients were examined. Anatomical and functional changes seen after treatment were evaluated.Results:One (16%) of the cases was female and five (83%) were male. The mean age was calculated as 36.3±24.9 years (range 11–73 years). The mean follow-up period of the cases after detecting CNV was determined as 26 months (range 6–168 months). Best corrected visual acuities were 0.65±0.39 logMAR (1.0–0.2 logMAR) when CNV was detected and 0.42±0.25 logMAR (limits 0.7–0.2 logMAR) at the end of the follow-up. Photodynamic therapy was applied to one (17%) of the cases, and intravitreal anti-vascular endothelial growth factor (anti-VEGF) treatment was applied to the others (83%). The mean number of intravitreal injections was found to be 3±1.37 (range 2–4).In all cases, intraretinal fluid (IRF), subretinal fluid (SRF), external limiting membrane, and irregularities in inner retinal layers were observed in the initial OCT examinations. In all cases, it was observed that the IRF regressed with treatment and SRF continued in five eyes. Hypertrophic outer retinal scarring developed in all cases.Conclusion:In cases with CNV due to BVMD, regression in CNV activity was achieved with intravitreal anti-VEGF injection treatment. The IRF is a marker that can be used both in the diagnosis and treatment monitoring of CNV associated with BVMD.