Pseudoexfoliation (PEX) syndrome is a common ocular disease that also affects the cornea. A case of clinical PEX syndrome, studied by in vivo corneal confocal microscopy is reported. The morphological analysis of the confocal images demonstrated hyper-reflective deposits and several dendritic cells in the basal epithelial layer. A fibrillar subepithelial structure was also found. The endothelial layer showed cell anomalies (polymegathism and pleomorphism) and hyper-reflective small endothelial deposits. Confocal microscopy is an in vivo imaging method that may provide new information on corneal alterations in PEX, and detect early corneal features.