Increasing pathophysiologic evidence indicates that exfoliation syndrome, a systemic process involving various visceral organs, frequently manifests itself first in the eyes as exfoliation of the lens and glaucoma. Exfoliation syndrome occurs when several tissues synthesize an abnormal basement membrane protein. This protein may obstruct the trabecular meshwork in the eye and cause glaucoma. We present a case of exfoliation syndrome associated with angle-closure glaucoma in a 57-year-old male patient from Taiwan. After peripheral iridectomy and topical antiglaucoma medications, intraocular pressure in both eyes was well controlled in the range of 15 to 20 mmHg. The mechanism of the glaucomatous optic neuropathy in this patient was probably caused by chronic angle-closure glaucoma superimposed with the exfoliation syndrome.