A case of Wilson disease (WD) combined with keratoconus (KC) is described. A 30-year-old male diagnosed with WD presented to Ophthalmology Department due to progressive bilateral vision loss. Biomicroscopy revealed copper depositional ring and mild central corneal ectasia in both eyes. The patient had essential tremors and mild speech disturbance. The keratometric values were K1 = 45.94 diopters (D), K2 = 49.10 D in the right eye, and K1 = 47.14 D, K2 = 51.22 D in the left eye. The maximal elevation points on the posterior elevation maps were 98 mm for the right eye and 94 mm for the left eye. The typical KC pattern was seen on corneal topography bilaterally. Based on these findings, the patient was diagnosed with KC, and corneal cross-linking treatment was recommended. WD rarely occurs in combination with KC, and only two cases have been reported; this is the third case of WD combined with KC so far.