Abstract
Familial gingival fibromatosis is a rare hereditary condition due to chromosomal abnormality which can occur as an isolated disease or as part of a syndrome and has an incidence of 1:350 000. This condition leads to esthetic, functional, psychological and masticatory disturbance of the oral cavity. Here, we present a case of 21-year-old female with severe enlargement of gums in maxilla and mandible. Deciduous teeth were erupted at normal age but the permanent teeth in the oral cavity were not erupted. Her grandmother, father and younger sister were also affected with the same condition. Incisional biopsy from the chronic ulcerated gingiva showed squamous cell carcinoma. Patient was referred to other cancer institution, where metastases to lung, bone and lymph node was detected on CT scan. The diagnosis was made based on clinical examination, family history and histopathological examination.