The exfoliation or pseudo-exfoliation syndrome (PE) is very rare in China. The clinical and histopathologic features in 13 patients were that the loss of pigment at the pupillary ruff and dense pigmentation on the trabecular meshwork were early signs easily overlooked, and the dandruff-like particles at the pupillary margin and greyish white ring at the anterior periphery of the lens were typical findings. Histopathologic study demonstrated that the PAS-positive amorphous material under light microscopy were fibrils in structure and accumulated at various sites in the anterior segment of the eye.