Verruciform xanthoma (VX) is a rare, benign, mucocutaneous, verrucous, papillary lesion. This paper retrospectively summarizes clinical and pathologic features of 32 vulvar verruciform xanthoma reported from China and abroad. The skin lesions are generally single, mainly in labia minora, clitoris and fourchette with partly extending to the groin, buttocks and anus. The possible inducing factors include long-term scratching, local itching, severe lymphedema or lymphangioma circumscriptum. Severe cutaneous trauma and chronic inflammation may be the main causes. Clinically, it can easily be misdiagnosed as condylomata acuminata, squamous cell carcinoma, bowenoid papulosis, etc. It is reported to be related to underlying disorders. The main treatment is complete resection.