Purpose: To identify and describe the in-vivo microstructural changes in the cornea of patients with pre-Descemet’s membrane corneal dystrophy. Methods: Ten patients (20 eyes) were examined in the Laser Microsurgery Centre in Warsaw. The ophthalmic examination, including visual acuity and slit lamp biomicroscopy, was carried out. Because of suspicion of corneal dystrophy, in vivo confocal microscopy of the cornea was also performed. Results: Biomicroscopy revealed bilateral, dust-like fine opacities in the deep parts of the corneal stroma. Confocal microscopy showed pleomorphic structures (enlarged keratocytes) containing dense, hyperreflective, granular inclusions in the posterior stroma, next to the Descemet’s membrane. In 3 patients (6 eyes) punctate particles were seen extracellularly also in the mid stroma. Superficial and basal epithelial layers, anterior stroma and endothelium cells appeared normal. Conclusions: 1. In vivo confocal microscopy findings of cornea with pre-Descemet’s membrane dystrophy are characteristic. 2. In vivo confocal microscopy is a useful tool in diagnosing rare corneal dystrophies in cases where classical examination methods give inconclusive results.