Autosomal recessive bestrophinopathy (ARB) is a rare inherited retinal dystrophy caused by mutations in the BEST1 gene, characterized by subretinal accumulation of vitelliform material and retinal pigment epithelium dysfunction. We report the case of a 7-year-old child presenting with progressive visual loss in the right eye. Fundus examination revealed bilateral vitelliform lesions associated with multiple extramacular deposits, with marked asymmetry between both eyes. Multimodal imaging, including optical coherence tomography (OCT), fundus autofluorescence, and fluorescein angiography, demonstrated subretinal hyperreflective vitelliform material, pseudoserous retinal detachment, cystoid macular edema (CME), and subretinal fibrosis in the right eye. Electro-oculography showed a markedly reduced Arden ratio, while electroretinography revealed both photopic and scotopic dysfunction. Genetic analysis confirmed a mutation in the BEST1 gene, establishing the diagnosis of autosomal recessive bestrophinopathy. No specific treatment was initiated, and regular follow-up was recommended. This case highlights the phenotypic variability of ARB and emphasizes the importance of multimodal imaging and genetic testing for accurate diagnosis and management.