A patient with white sponge nevus involving the buccal mucosa, labial mucosa, and soft palate is reported. The salient clinical and histological features of the disease were discussed. This diagnosis should be considered when bilateral white lesions of buccal mucosa are encountered. Early onset, a familial history, and the asymptomatic nature are key features to the disease. If historical data and clinical features are suspect, an incisional biopsy is adequate for diagnosis.