Transient receptor potential vanilloid 3 (TRPV3) is a warm-temperature sensitive calcium-permeable nonselective cation channel that is activated by innocuous heat above 33℃. TRPV3 is involved in skin barrier repair, pruritus and inflammation, and its genetic mutations lead to a rare genetic disease called Olmsted syndrome characterized by bilateral mutilating transgredient palmoplantar keratoderma (PPK), periorificial keratotic plaques and chronic pruritus. These findings indicate that TRPV3 is likely to serve as a drug target and selective inhibition of TRPV3 may hold therapeutic potential for treatment of pruritus and skin-related diseases. This review outlines recent progress in the structure and function of TRPV3,as well as its pathological roles in skin diseases.