Hedgehog signaling pathway plays an important role in regulating the development of endocrine glands. Desert hedgehog (Dhh) has become a recent focus for its regulation of testis development, especially of Leydig cells. Dhh, as a Sertoli cell product, regulates the proliferation and differentiation of Leydig cell lineage and functions to secrete testosterone through a paracrine signaling mechanism. Testosterone, as the most important sex hormone of the male, plays a critical role in testis development, spermatogenesis and maintenance of normal masculinization. Therefore, normal Dhh signaling pathway ensures normal spermatogenic function. Researches on the Hedgehog signaling pathway in the testis have a potential significance for studying the pathophysiological mechanisms of androgen deficiency and dyszoospermia, as well as for the clinical treatment of these diseases.