The Klippel-Trénaunay syndrome (KTS) was first described by Klippel and Trénaunay in 1900. It is characterized by the triad of hemihypertrophy of soft and hard tissue, naevus flammeus and venous varicosity in the affected area. Though all oral tissues may be affected, only 5% of KTS show manifestations in the head and neck region. Only three cases are described with an oral manifestation, showing gingival overgrowth clinically and histologically corresponding to a pyogenic granuloma. It is still uncertain whether the combination of gingival fibromatosis and KTS is significant or coincidental. We report about a 25-year-old patient with KTS and recidivous gingival fibromatosis, clinically and histologically corresponding to an epulis fibromatosa in a case report. It is suggested that this occurrence is significant.
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