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Laugier-Hunziker syndrome is an acquired macular hyperpigmentation of the oral and genital mucosa, often associated with longitudinal melanonychia. We report a case of longitudinal melanonychia on fingernails and toenails, hutchinson's signal and lenticular pigmentation of her lips and oral mucosa. Histopathological analysis is typical and had a broad differential diagnosis. This is a rare and benign entity, which should be included in the differential diagnosis of mucocutaneous hyperpigmentation.