OBJECTIVE The presentation and initial response to treatment of consecutive patients with exfoliation glaucoma (PXE) and primary open-angle glaucoma (POAG) were evaluated prospectively. METHODS Forty-six consecutive newly diagnosed patients with POAG and PXE were included in a prospective study that evaluated the initial clinical course and treatment results. The two groups were age- and race-matched. RESULTS This study found no difference in optic disc parameters at presentation between patients with POAG (n = 22) and those with PXE (n = 24) (P > .05). However, the presenting mean defect was significantly worse in patients with PXE versus those with POAG (P = .0038), although the loss variance was similar (P > .90). The mean presenting intraocular pressure (IOP) for patients with PXE (32.0 +/- 5.2 mm Hg) was greater than that for patients with POAG (27.1 +/- 4.4 mm Hg) (P= .0025). Additionally, patients with PXE required more treatment steps to control IOP (P = .005). Thirteen of 24 patients with PXE versus 3 of 22 patients with POAG (P = .016) required either laser or conventional surgical techniques to reduce IOP. CONCLUSIONS This study shows that patients with PXE have greater visual field loss and have more difficulty gaining control of IOP on presentation than patients with POAG.