There is still considerable controversy concerning the role of androgen therapy for hypoactive sexual desire disorder in females. Clinical trials have shown that exogenous testosterone therapy improves arousability, sexual desire and fantasy, frequency of sexual activity and orgasm, and satisfaction and pleasure from the sexual act. We report our experience of 36-year-old fragile X carrier female presented to our department with low sex drive and subjectively poor arousal for the past 1 year. We decided to treat her with local testosterone in a pump form (1% 5 g twice a week). She responded very well to the treatment, and in 3 weeks her libido and sexual functions improved dramatically. We decided to continue the same treatment for almost 1 year following up her free and total testosterone, complete lipid profiles, liver functions and complete blood picture every 3 months. During the whole course of treatment, she had mild facial acne, which was resolved in 2 months after completing treatment with testosterone. This is our first and only case of this syndrome with low sexual desire disorder treated with local testosterone in a pump form. Based upon our 1 year follow-up, low-dose testosterone is a safe and effective approach in treating hypoactive sexual desire disorder in a fragile X carrier female, provided the patient is monitored every 3 months for blood counts, lipid profiles, liver functions and free and total testosterone levels.