Azoospermia could be either non-obstructive azoospermia (NOA) or obstructive azoospermia (OA). Clinical assessment (testis consistency & volume), laboratory testing (FSH), and genetic testing (karyotype, Y chromosome microdeletion, or specific genetic testing for hypogonadotropic hypogonadism) are used to distinguish NOA (which includes primary and secondary testicular failure) from OA. The treatment of NOA is still empirical. For hypogonadotropic hypogonadism, gonadotropin therapy is the only particular indication that consistently improves semen analysis and rates of conception. The typical treatment consists of gonadotropins (hCG and rFSH) combined, with GnRH therapy maintained for non-responders. Although there is a paucity of level I clinical data, drug therapy combining aromatase inhibitors and gonadotropins may be able to improve outcomes for men who need surgical sperm retrieval. Many trials have been tried for treating NOA, although none of these trials has consistently shown efficacy. This review offers a current overview of the causes, treatments, and management of non-obstructive azoospermia.