Signaling pathways play an important role in receiving and transmitting information in the cell and in the intercellular communication, and are essential for the normal function of the organism. Thus, the highly conserved Hedgehog (Hh) signaling pathway, which was discovered in the fruit fly (lat. Drosophila melanogaster), is important for the transmission of information related to the proper development of organs and body plan during vertebrate and invertebrate embryogenesis. It also plays a role in maintaining homeostasis and regeneration of adult tissues. It consists of a cascade of signaling molecules: Hh proteins which are extracellular ligands, Patched receptor, Smoothened protein, Kif7, Sufu and Gli transcription factors. Their activity must be precisely regulated in order to allow proper cell differentiation and proliferation to occur. Abnormalities associated with weak or disabled signaling during embryonic development are associated with birth defects, while increased activity of the signaling pathway in adult tissues leads to the formation of certain types of tumors. Today, there are many drugs for the treatment of tumors which act as Hh signaling pathway inhibitors