This systematic review evaluates the efficacy and safety of l-arginine alone or in combination for the treatment of women with hypoactive sexual desire disorder (HSDD) or related conditions, such as female sexual interest/arousal disorder and female sexual arousal disorder. Medline, Embase, International Pharmaceutical Abstracts, Science Direct, and the Cumulative Index to Nursing and Allied Health Literature were searched using keywords “arginine”, “Lady Prelox”, “ArginMax”, “Stronvivo”, “Ristela”, “hypoactive sexual desire disorder”, “female sexual interest arousal disorder”, “female sexual arousal disorder”, “sexual dysfunction”, “sexual behavior”, “dyspareunia”, “libido”, and permutations thereof. Relevant records were retained if they were primary literature, conducted in women with HSDD or related conditions, and published as full text in English. Five randomized controlled trials and two nonrandomized studies met eligibility criteria. Six of the seven studies reported either an increase in the total mean Female Sexual Function Index score or significant increases in multiple domains therein. One study assessed vaginal pulse amplitude and found a statistically significant increase in a combination treatment group compared to placebo. No significant side effects were reported. Four of seven studies had potential risk-of-bias concerns per Cochrane assessments. This systematic review found that combination products containing l-arginine in the form of ArginMax or Lady Prelox may be considered for the treatment of HSDD and related conditions in women regardless of age.