Purpose: To present longitudinal, multimodal imaging findings of leptovitelliform maculopathy, an underrecognized condition within the spectrum of adult vitelliform lesions. Methods: A single case was evaluated over 5 years. Results: A 70-year-old man presented with vitelliform lesions accompanied by subretinal drusenoid deposits/reticular pseudodrusen and a thin choroid (leptochoroid). Multimodal imaging demonstrated progressive collapse of the vitelliform lesions, followed by extensive and worsening outer retinal atrophy. Conclusions: Leptovitelliform maculopathy may demonstrate progressive collapse of vitelliform lesions, ultimately leading to significant outer retinal atrophy. Longitudinal multimodal imaging is valuable in characterizing this aggressive and underrecognized disease entity.