Genome editing is a cutting-edge technology that enables to modify the target gene using programmable site-specific nucleases, such as TALENs and CRISPR/Cas9. Currently, cell and animal models of human diseases have been competitively created throughout the world, because genome editing technology paved the way for genetic modifications even in cells and organisms that had been difficult to manipulate the genome. In this review, we introduce the basic principles and current situations of genome editing with programmable nucleases.