Purpose: To describe the clinical and in vivo confocal microscopic findings of the cornea in 2 patients with Bietti crystalline corneoretinal dystrophy using Heidelberg Retina Tomograph II Rostock Cornea Module. Methods: Two women 25 and 33 years of age underwent ophthalmologic assessment, including fundus photography, optical coherence tomography, fluorescein angiography, electroretinography, and in vivo confocal microscopy. Results: Slit-lamp examination revealed crystalline deposits at the superior limbus of the cornea in both of the subjects. Fundus examination disclosed numerous glistening yellowish white crystalline deposits scattered throughout the posterior pole and midperipheral retina, retina pigment epithelium and choriocapillaris atrophy; pigment clumping; and retinal scarring. Optical coherence tomography demonstrated hyperreflective red and white areas corresponding to the crystalline deposits in the retinal pigment epithelium-choriocapillaris complex. In vivo confocal microscopy of the superior paralimbal area showed randomly oriented needle-shaped or rod-shaped crystals up to 40 μm in length and 4-8 μm in width in the epithelium and the stroma. In other areas, the epithelium, stroma, and endothelium had normal appearance with no deposits. Conclusions: In vivo confocal microscopy is a noninvasive examination technique that shows clearly the corneal crystals located mainly in the superior paralimbal area in Bietti crystalline corneoretinal dystrophy, which can easily be missed even by an experienced ophthalmologist and therefore may aid further in the diagnosis. Copyright © 2010 by Lippincott Williams & Wilkins.