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The case of a 13-year-old female patient is here described. Frontal and temporal lesions\nappeared at 4 years of age, followed by smaller malar and chin lesions since age 5. Her\nmother referred hypochromic maculae on dorsum present from birth. In childhood, she had\none episode of seizure associated with fever, with normal EEG. There was no family\nhistory of similar skin lesions.\nIn CT of the skull, multiple amorphous subependimary calcifications were present. In\nthoracic and lumbar spine CT, partial defect in fusion of the posterior neural arch was\nfound at the T11, T12, S1 and L5 levels.\nOn examination, she had multiple pinkish-brown, shiny papules, about 2 mm in diameter in\nthe malar region and chin, brownish frontal and pre-auricular plaques and multiple\nhypochromic stains on the trunk, including ash leaf spots (, and ). The frontal and pre-auricular lesions are\nprobably fibrous plaques, classified as angiofibromas; the patient did not allow a\nbiopsy, so other possible diagnosis such as melanocytic naevi, verrucous naevi and even\nShagreen patch with unusual localization should be considered.