A 39-year-old man with pseudoxanthoma elasticum who had a loss of vision was examined using digital imaging. Spectral domain optical coherence tomography (SD-OCT) detected subfoveal deposits that coincided with hyperautofluorescent areas on fundus autofluorescence imaging. These deposits seemed to cause the patient's rapid visual loss because no angioid streaks or choroidal neovascularization were present in the fovea. These deposits were not observed on fundus and infrared photography. SD-OCT combined with fundus autofluorescence imaging may be a useful tool for diagnosis of the atypical deposits in patients with pseudoxanthoma elasticum, which could lead to visual loss.