Two hundred fifty-seven patients with biopsy-proven dysplastic nevus syndrome (DNS) were evaluated ophthalmologically and compared with 264 non-DNS patients, who acted as a control population. A statistically significant increase in the percentage of the DNS patients versus controls harboring conjunctival nevi (8.56% vs. 1.14%), iris nevi (24.90% vs. 9.47%), and choroidal nevi (14.40% vs. 5.30%) was encountered. The number of iris nevi found per patient was also greater statistically for the DNS group. Also encountered in the DNS group was presence of multiple nevus types in the same patient (iris and choroidal, iris and conjunctival, conjunctival and choroidal); 3 patients had all three nevus types present, and 4 patients had two choroidal nevi per eye. No patient in the control group had more than one nevus type present per eye.