OBJECTIVE To report the cross-sectional structure of the retina and choroid in eyes with adult-onset vitelliform macular dystrophy as obtained by optical coherence tomography (OCT). METHODS Seven patients with adult-onset vitelliform macular dystrophy and one patient with Best disease were examined by fundoscopy, fluorescein and indocyanine green angiography and OCT. Three patients underwent also electro-oculography. RESULTS 1. Seven cases with adult-onset vitelliform macular dystrophy showed a well-circumscribed elevation of a highly reflective band, corresponding to the retinal pigment epithelium 2. In these 7 patients, the space below this band was inhomogeneous and moderately reflective. 3. Four cases out of 7 had a well defined posterior boundary. 4. The patient with Best disease disclosed a different aspect on OCT, although the contour of the lesion was similar to the others. CONCLUSIONS Optical coherence tomography disclosed the structure of the vitelliform lesion in vivo and could be helpful for its pathological interpretation.