To evaluate the clinical usefulness of a spectral-domain ultra high-resolution anterior segment optical coherence tomography (UHR OCT) in examination, diagnosis, and management of various anterior corneal dystrophies and degenerations. Noncomparative case series. Fifty-nine eyes of 38 consecutive patients were enrolled in the study and included 28 eyes of 14 patients with anterior corneal dystrophies, 21 eyes of 19 patients with anterior corneal degenerations or neoplasia, and 10 eyes of 5 patients with normal corneas. Subjects were imaged using a novel custom-built UHR OCT. Images were used to evaluate and describe the characteristics of anterior corneal dystrophies and degenerations. Nineteen patients underwent surgical management, and those histopathologic specimens were analyzed and correlated with the UHR OCT images. Comparison of clinical findings, UHR OCT images, and corresponding histopathologic specimens. The UHR OCT provided clear delineation of corneal anatomic features and pathologic corneal deposits in most cases. The characteristics and depth of these deposits are illustrated and can be localized to specific layers of the cornea. When available, there was significant correlation between UHR OCT images and histopathologic features, providing a noninvasive confirmation of the clinical diagnosis. Ultra high-resolution OCT is an innovative technique to perform in vivo optical biopsies and a promising research and clinical tool for the evaluation of corneal pathologic features in a noninvasive manner. The future use of this novel technology will evolve and increasingly is becoming a vital tool in the clinical and surgical management of corneal diseases.