The review analyzes the current state of experimental studies on the ability to obtain and cultivate stem cells from the nail organ and their possible involvement in the regeneration of a limb. It has been known that the nail unit consists of a pool of undifferentiated cells which provide sustained growth and nail repair throughout life. But, nowadays the issue of stem cell niche localization in the nail organ remains unresolved. Also, researchers demonstrated involvement of these cells in the restoration of amputated limbs, in particular, through activation of certain signaling pathways (Wnt, BMP, Notch), and epithelial-mesenchymal interactions, but the detailed mechanism of this process is poorly understood. It is supposed that the nail organ has two sources of undifferentiated cells of different origin: the proximal nail fold and the dorsal part of the nail matrix (K15+, K19+, PHLDA1+); and onychodermis (CD10+, CD34–). However, these markers are not generally accepted, so the search for markers combinations for exhaustive and complete characterization of stem cells from the nail organ continues.