Purpose To examine changes in visual acuity (VA) and refraction in non-glaucomatous patients with unilateral exfoliation syndrome (EXS). Methods The best corrected values for VA (Snellen acuity cards) subjectively adjusted for refraction, and IOP were measured, and the development of lens opacities was examined in 46 non-glaucomatous patients with unilateral EXS. Results After five years the rate of conversion to bilateral exfoliation was 22% and to exfoliative glaucoma 30%. There was a significant decrease in VA in the exfoliative (E) eyes (median; QI, QIII, range: 1; 0.8, 1, 0.4-1.3 vs. 0.55; 0.4, 1, 0.05-1.4, p<0.0001) and the fellow, initially non-exfoliative (NE), eyes (1; 0.9, 1, 0.3–1.3 vs. 0.7; 0.5, 0.9, 0.1–1.4, p<0.0001) and a significant myopic change in refraction in the E eyes (+1.02 ± 2.48 vs. +0.11 ± 3.06, p=0.0001) and the NE eyes (+0.99 ± 2.25 vs. +0.43 ± 2.55 D, p<0.01). At study entry the difference in refraction between the fellow eyes (refraction in the NE eye – refraction in the E eye) was −0.27 ± 1.00D. After five years it was +0.32 ± 1.44 (p 0.016), reflecting greater myopic changes in the E eyes. The main type of lens opacification was nuclear sclerosis. Conclusions In five years, significant decreases in VA and myopic shifts in refraction occurred in the E and fellow eyes. The E eyes showed significantly greater myopic changes than the fellow eyes; the cause was clearly nuclear sclerosis, which must be taken into account in the long-term management of patients with EXS.