A 9-year-old boy was diagnosed with fundus flavimaculatus in his left eye. The boy's fellow eye was enucleated at 16 months of age for retinoblastoma. The authors reviewed the material submitted for histopathologic examination and found that the retinal pigment epithelial cells demonstrated increased autofluorescence and increased reactivity to periodic acid-Schiff staining. Many cells had their melanin granules displaced toward the cell apex. The retinal pigment epithelial changes are consistent with previous histopathologic findings in fundus flavimaculatus and imply that the structural changes are seen in early life.