Menkes disease, also known as the „kinky hair disease” is a progressive neurodegenerative disorder, which is connected with the disturbances of the copper absorption. The disease is caused by the lack or disability function of the copper transporting protein, (type P of ATPase), which is normally responsible for the maintenance of an appropriate level of the copper in the serum (there is a mutation of ATP7A gen, which is located on the locus Xq13.3). This results in the excessive accumulation of the copper in the intestinal wall, proximal renal tubular, pancreas, skeletal muscles and placenta, as well as its deficiency in the brain cells, the liver and connective tissue cells. The copper taken with food is not absorbed from the gastrointestinal tract.