The ubiquitin pathway (UP) is involved in regulation of many essential cellular processes usually by the degradation of regulators of these processes. For example the UP is involved in regulation of cell cycle, proliferation, differentiation, organogenesis, development, and signal transduction in the lens and retina. A functional UP has also been documented in the cornea. Upon aging or exposure to stress there is an accumulation of damaged proteins, including ubiquitinated proteins, in the lens and retina. Some of these proteins may be cytotoxic. Thus, an active UP may be required to avoid such age and disease-related accumulation of damaged proteins. In this review we will explain the biochemistry of the UP and we will document the most important studies regarding UP function in the lens, retina and cornea.