Purpose: To analyze biometric changes and prevalence of refractive in patients with neurofibromatosis type 1 (NF1). Methods: Retrospective, case-controlled study involving patients affected by NF1 and healthy control subjects. Data on biometric measurements such as axial length (AL), central corneal thickness (CCT), anterior chamber depth (ACD), lens thickness (LT), keratometry (K1 and K2) values, and white-to-white (WTW), obtained by use of optical low-coherence reflectometry on a Lenstar LS 900® (Haag-Streit AG, Switzerland) were collected and analyzed. Cycloplegic refractions were then performed. Results: Overall, 166 eyes of 83 patients diagnosed with NF1 (mean age 21.6 ± 9.8) were enrolled and compared with 178 eyes of age-matched healthy subjects (mean age 22.6 ± 6.6). One hundred sixty-six (22.8%) and 33 of 178 (18.5%) eyes were myopic in NF1 patients and healthy subjects, respectively. The prevalence of hyperopia in the NF1 group was 12 of 166 (7.2%) whereas in the healthy control group was 14 of 178 (8.9%). Twenty-nine of 166 (17.4%) and 34 of 178 (19.1%) eyes presented astigmatism in NF1 and control group, respectively. These differences were not statistically significant (p-values > 0.05). Refractive errors such as myopia, hyperopia, and astigmatism were similar between the two groups. The difference of AL, CCT, ACD, LT, K values, and WTW were no statistically significant between the two groups (p-values > 0.05). Conclusion: Refractive errors and ocular biometric parameter seem not to be an addition findings of NF1. © The Author(s) 2020.