Background. Adult-onset vitelliform macular dystrophy is one of the pathologies that successfully masks as age-related macular degeneration. Purpose. To assess the incidence of adult-onset vitelliform macular dystrophy and its clinical manifestations. Material and methods. We analyzed frequency cases of adult-onset vitelliform macular dystrophy among 1000 patients aged 40 years and older who applied with various eye complaints. Results. Adult-onset vitelliform macular dystrophy was found in 2 patients – men 43 and 66 years old. In the macular area of both eyes of the 1st patient, rounded yellow lesions in the foveola, local detachment of neuroepithelium, optical dense material deposit on the outer segments of photoreceptors were detected (vitelliruptive stage of adult-onset vitelliform macular dystrophy). On the left eye of the 2nd patient in the foveola, a single rounded yellow focus of 200 µm was determined, optical dense vitelliform material presence between the layers of the pigment epithelium and neuroepithelium without signs of choroidal neovascularization was detected (vitelliform stage of adult-onset vitelliform macular dystrophy). Conclusion. The incidence of adult-onset vitelliform macular dystrophy among patients with various ocular pathologies was 0.2 %. Morphological changes in the macula consist in presence of deposits of vitelliform material, localized between the layers of neuroepithelium and retinal pigment epithelium. Keywords: adult-onset vitelliform macular dystrophy; age-related macular degeneration; optical dense vitelliform material