Hereditary hemorrhagic telangiectasia(HHT)is an autosomal dominant inherited disorder affecting blood vessels of the skin, mucous membranes and viscera. A definite diagnosis of HHT is made in the presence of at least three of the Curaçao criteria: (1)epistaxis,(2)telangiectasias,(3)visceral lesions, and(4)a family history. We describe a case of upper gingival carcinoma in a patient with HHT. A 79-year-old woman visited our department because of an upper gingival mass. A biopsy specimen revealed

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... squamous cell carcinoma. Preoperative examination revealed consistency with three of the above criteria: (2)skin and mucosal telangiectasias,(3)gastrointestinal telangiectasia and pulmonary and hepatic arteriovenous malformations(AVMs) , and(4)mucosal telangiectasia and pulmonary AVM in her daughter. HHT was definitely diagnosed. Tumorectomy was scheduled; however, HHT-associated complications such as bleeding, hypoxemia, cerebral thromboembolism, and AVM rupture were expected. Tumor resection by partial maxillectomy and reconstruction with a buccal fat pad were performed under general anesthesia. The postoperative course was uneventful, and follow-up at 1.5 years showed no evidence of tumor recurrence or metastasis. We conclude that recognition of hemorrhagic tendencies and evaluation of systemic AVMs are critical before surgical treatment in patients with HHT.