A 17-year-old white boy was seen at the outpatient clinic of the Discipline of Stomatology, Sa˜o Jose´dos Campos Dental School-UNESP, complaining of the presence of nodules in the mouth, nasal, and palpebral mucosae, unaltered since birth. Clinical examination revealed painless mucosal nodules of variables sizes on the lips, buccal mucosa, and lateral borders of the tongue and palate, whose color was similar to that of the adjacent mucosa (Fig. 1). Papules were also present in the nasal mucosa and on the left lower eyelid (Fig. 2). The lips had been submitted to cosmetic surgery years before but without histopathological analysis of the removed material. Physically, the patient exhibited a tall stature and evidently elongated upper and lower limbs, in addition to manifestations of scoliosis. The patient was otherwise apparently well with no systemic symptoms and had no other dermatologic lesions.A biopsy was taken from one of the nodules located on the left lateral border of the tongue and sent for histological analysis (Fig. 3).