Dental enamel is the most mineralized tissue all over the body. The enamel development begins with the organic matrix secretion. The enamel extracellular matrix mainly consists of amelogenins and enamelins. As the enamel maturation progress the organic matrix undergoes proteolitic degradation. The remnants, which remain after the degradation are subsequently withdrawn and replaced with hydro- xyapatites crystals which form the enamel prisms. The enamel secretion and maturation can be modified by fluoride ions supplemented per os. It is extremely difficult to establish the daily optimal dose of fluoride which if exceeded leads to disturbances in the enamel mineralization and the fluorosis origin. The mechanism of the dental fluorosis origin has not been completely explained yet. Nevertheless the available data confirm that dental fluorosis is a side effect of disturbances in the proteolitic degradation of the proteins that form the enamel extracellular matrix. In the paper we depicted the proteins which are the main components of the enamel extracellular matrix as well as the influence that fluoride exerts on their metabolism, which can result in the fluorosis.