Purpose: The purpose of the study was to evaluate the ocular surface and corneal sensitivity in patients with lamellar ichthyosis (LI). Methods: Eleven eyes of 11 patients with LI (Group 1) and 11 eyes of 11 healthy individuals (Group 2) were enrolled into this cross-sectional study. Detailed ophthalmological examination along with ocular surface fluorescein staining with Oxford scoring, tear film break-up time, Schirmer 1 test, ocular surface disease index (OSDI) score assessment, and evaluation of corneal sensitivity with Cochet-Bonnet esthesiometer was performed. Results: The mean ages of Group 1 and Group 2 were 24.54±10.22 years (range, 11–37) and 26±7.53 years (range, 16–40), respectively (p=0.764). Male/female ratio was 5/6 in Group 1 and 4/7 in Group 2. Mean tear film break-up time and the corneal sensitivity of the superior and inferior region of cornea were lower (p=0.00008; p=0.019; and p=0.006, respectively), and OSDI and Oxford scores were significantly higher in Group 1 (p<0.00001 and p=0.002, respectively). No significant difference in terms of Schirmer 1 test and corneal sensitivity of central, temporal, and nasal regions was detected (p>0.5). Conclusion: LI is not only associated with evaporative type dry eye but also decreased corneal sensitivity of peripheric cornea. Therefore, to prevent uninvited complications, LI patients should be examined for dry eye regularly, even if they do not have any complaints.