Background Vortex keratopathy, arising as a side effect of several medications, is characterized by golden-brown deposits in the cornea. Methods A 41-year-old woman treated for sarcoidosis with hydroxychloroquine therapy and suffering from vortex keratopathy was examined by in vivo confocal microscopy. Scans of both corneas were performed. Results By slit lamp examination, the left but not the right eye showed a golden-brown deposit throughout the cornea. In vivo confocal microscopy revealed the presence of highly reflective, dot-like intracellular inclusions concentrated in the basal epithelial layer. They were also detected within the anterior and posterior stroma, but not within the endothelium. In regions of the anterior stroma, devoid of inclusions, hyperreflective ramified keratocytes were observed, forming an extended interconnecting network. Conclusion In addition to the granular deposits, in vivo confocal microscopy revealed hyperreflective, possibly phagocytic keratocytes.