The Hedgehog (Hh) signaling pathway plays a key role in the embryonic development, which is formed by the Hedgehog(Hh) , membrane-receptor complex Patched (Ptch) and Smoothened (Smo), multifunctional transcription factors Glioma-associated oncogene homolog (Gli) and other signal molecules. The Hh signaling pathway controls a variety of developing processes, however, no or less expression is observed in the normal pancreas tissues.The mutational activation of the Hh signaling pathway is associated with tumorigenesis and metastasis of the pancreatic cancer. The aim of this review is to present a brief overview of the Hedgehog signaling pathway in the mechanism of the tumorigenesis of the pancreatic cancer.