Peutz-Jeghers syndrome is an eponym for circumscribed mucocutaneous melanosis in association with gastrointestinal polyposis. Irregular pigmented macules of varying size and color can be found on the perioral skin, lip vermilion border, buccal mucosa, palate, and tongue. Previous treatments, such as surgical excision, cryosurgery, electrodesiccation, dermabrasion, and carbon dioxide or argon laser ablation commonly result in incomplete removal, scarring, or changes in normal pigmentation. The Q-switched ruby laser used at 694 nm, a wavelength well absorbed by melanin relative to other optically absorbing structures in skin, causes highly selective destruction of pigment-laden cells. In addition, the 20-nanosecond pulse duration produced by this laser approximates the thermal relaxation time for melanosomes, thereby confining the energy to the target. The Q-switched ruby laser produces clinically significant fading of mucocutaneous melanosis in association with Peutz-Jeghers syndrome without complications often seen with other therapeutic modalities.