OBJECTIVE To systematically review the management of sexual dysfunction (SD) due to central nervous system disorders. METHODS The review was done according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Studies were identified independently by two reviewers using electronic searches of MEDLINE and OVID (from January 2004 to August 2014) and hand searches of reference lists and review articles. RESULTS In patients with central nervous system disorders, neuro-urological assessment is recommended for both genders before starting any treatment for SD. For men, blood sexual hormones evaluation is the main investigation performed prior to phosphodiesterase type 5 inhibitors (PDE5Is) treatment, whereas there is no consensus on routine laboratory tests for women. PDE5Is are the first-line medical treatment for men, with the most robust data derived from patients with spinal cord lesion assessed by validated questionnaires, mainly the International Index of Erectile Function-15. There is no effective medical treatment for SD in women. Sacral neuromodulation for lower urinary tract dysfunction may improve SD in both genders. CONCLUSIONS Although SD is a major burden for patients with central nervous system disorders, high-evidence level studies are rare and only available for PDE5Is treating erectile dysfunction. Well-designed prospective studies are urgently needed for both genders.