Abstract:  White sponge nevus is a rare, autosomal-dominant disorder that affects the noncornified stratified squamous epithelia. Clinically, the presence of white, spongy plaques mostly in the buccal, labial, and gingival mucosa and the floor of the mouth characterize the lesions. The differential diagnosis of the lesion may be difficult and it is best diagnosed by biopsy. We report a case of white sponge nevus in the oral cavity of a 16-year-old boy and review of the literature.