Purpose <p>This study investigated topographic, aberrometric, and endothelial cell characteristics of the cornea in Fabry disease and compared them to healthy eyes.</p> Methods <p>This prospective, cross-sectional study included 21 patients with Fabry disease and 41 healthy volunteers in Fabry disease (FG) and control (CG) groups, respectively. After ophthalmological examinations, the topographic, tomographic, and aberrometric corneal data were acquired using a Scheimpflug–Placido disk system anterior segment analysis device. The corneal endothelial examination was conducted using a specular microscopy device, and results were compared. A <i>p</i>-values of &lt; 0.05 were considered statistically significant.</p> Results <p>No significant differences were noted between the age and gender of the groups (<i>p</i> &gt; 0.05). Central and thinnest corneal thickness were significantly lower in the FG than in the CG (<i>p</i> = 0.036 and 0.023, respectively). Although the aberrometric values of the cornea were usually higher in the FG, this difference was not significant. No significant differences were observed between the two groups regarding corneal endothelial data examined by specular microscopy. When the eye data with cornea verticillata in the FG were compared with those of the CG, anterior higher-order aberrations (HOA) were significantly higher in the FG (<i>p</i> = 0.047) than in the CG.</p> Conclusions <p>Cornea verticillata associated with Fabry disease can increase HOA in the eye.</p>