Globozoospermia is a rare and serious teratozoospermia, which is one of the important causes contributing to human male infertility. The assisted reproductive technique remains the only means for such patients to produce offspring. However, the pathogenesis of globozoospermia is not yet clear. In recent years, related studies have shown that some genes are connected with the onset of globozoospermia. This paper outlines the progress in the studies of pathogenicity genes, aiming to contribute to the molecular diagnosis and mechanism investigation of the disease.