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Some authors recommend anatomopathological examination for diagnostic confirmation, although others sustain that the diagnosis is only clinical and follow-up is necessary to investigate changes in size, shape, and color of the lesion.2,4 Although the number of documented cases allows no definitive conclusions, a clinical diagnosis of congenital melanotic macule of the tongue should be considered in the presence of the following characteristics: single or multiple lingual melanotic lesions, presence of a macular lesion at birth with proportional growth, and absence of family history of systemic conditions associated with mucosal pigmentation.2 For acquired cases, lingual melanotic macules, although rare, should be considered in the differential diagnosis of pigmented lesions of the oral mucosa