White sponge nevus is a rare autosomal dominant hereditary disorder manifesting upon physical examination as white symmetric, diffuse, thickened, corrugated or velvety plaques on the oral, esophageal or genital mucosa. They are also found to be bilateral and asymptomatic. The tissue changes can be present at birth or manifest during childhood or adolescence, and this disorder occurs in females more than in males. It is a benign condition since cases of malignancy have not been reported, and it does not require treatment. Therefore, it is important to establish a differential diagnosis to rule out other white lesions that occur on the oral mucosa, mainly those in which there is a risk of malignancy. A case of white sponge nevus is reported along with a discussion on the differential diagnosis and clinical management.