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Most melanotic macules are congenital, affect Caucasian individuals, may be single or multiple, have a diameter varying between 0.3cm and 3cm, tend to grow in proportion to the body growth, and are unrelated to any systemic condition.2,4 Their clinical features are not yet well-defined.1,2 The etiology of the disease still remains uncertain, but it is suggested that congenital cases may represent melanocyte hamartomas.2 Histology of the described cases of lingual melanotic macule reveals increased melanin in the basal cell layer, some pigment-laden macrophages in the dermis, normal number of melanocytes with no or a slight increase of melanin in their interior and variable degrees of hyperkeratosis.2 There are no nests of melanocytes, atypia, or alterations in the lingual tissue, and, in some cases, slight elongation of the epithelial ridges has been described.2 Also, according to Dohil et al., no similar histopathological alteration was identified in the non-affected lingual tissue.2 Our patient is an adult woman, which is uncommon when compared to other cases described

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Secondary carnitine deficiency is the more common condition resulting in low-levels of carnitine in healthy adults