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We excluded Peutz-Jeghers syndrome for the negative family history of polyposis of the colon, for the late onset of the spots, for the noninvolvement of the pseudomucosa and the perioral skin. Addison's disease, which can start with a hyperpigmentation of the oral cavity, has been excluded for the negativity of the hormonal examinations and the absence of other symptoms. We ruled out other causes of hyperpigmentation of the oral mucosa, such as medications, exposure to heavy metals, cigarette smoke for negative history. Lichen planus pigmentosum of the oral mucosa was excluded due to the absence of other signs like whitish reticulated papules and plaques and especially for the histology, characterized by the complete absence of inflammatory infiltrate and by the presence of melanin in the mucosal basal layer mucosa. In our case of Laugier-Hunziker syndrome lacked striated melanonychia, which is present in 44-60% of cases.