The constitution and the control of the ovarian reserve is of importance in mammals and women. In particular, the number of primordial follicles at puberty is positively correlated with the number of growing follicles and their response to gonadotropin treatments. The size of this ovarian reserve depends on genes involved in germ cell proliferation and differentiation, sexual differentiation, meiosis, germ cell degeneration, formation of primordial follicles, and on a potential mechanism of self-renewal of germ stem cells. In this review, we present the state of the art of the knowledge of genes and factors involved in all these processes. We first focus on the almost 70 genes identified mainly by mouse invalidation models, then we discuss the most plausible hypothesis concerning the possibility of the existence of germ cell self-renewal by neo-oogenesis in animal species and human, with a special interest for the role of corresponding genes in evolutionary distinct model species. All of the genes pointed out here are candidates susceptible to explain fertility defects such as the premature ovarian failure in human.