Neurofibromatosis 1 (NF1) is an autosomal dominant disease that is associated with multiple café-au-lait spots and neurofibromas. Low bone mineral density is frequent in people with NF1, and focal boney abnormalities are characteristic but uncommon features. Dental abnormalities can occur in association with oral neurofibromas but have not otherwise been described in people with NF1. Questionnaires regarding dental caries were sent to families that included at least one individual with NF1. Siblings with NF1 reported significantly more dental caries (mean ± SD, 8.1 ± 6.6) than siblings without NF1 in these families (5.5 ± 5.8, p = 0.019). Our findings suggest that dental caries occur more frequently than expected among people with NF1 and that individuals with this condition may require a modified dental care program.