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53歳女.20歳代前半より顔面, 頸部, 右腰部にドーム型の丘疹が出現し, 44歳時に皮膚科による生検の結果Birt-Hogg-Dube(BHD)症候群と診断された.近医で撮影した胸部レントゲン写真で異常陰影を指摘され, 呼吸器外科では腹部CTによる右腎腫瘍の指摘と共に異常陰影が肋骨転移と診断され受診した.腹部造影CTで右腎の腫瘍と, 骨シンチで多発性骨転移を認め, インターフェロン療法の効果を期待し右腎摘出術を施行した.術後, 骨転移に対し放射線治療及びインターフェロンを開始したが, 肺転移による呼吸不全の為術後約5ヵ月目に死亡した, Birt-Hogg-Dube (BHD) syndrome is a rare dermatological condition appearing with an autosomal dominant mode of inheritance. It was first reported in 1977 by Birt et al. and 28 cases have been reported since then. BHD syndrome is characterized by asymptomatic dome-shaped, skin-colored papules on the face and upper trunk. Recently, various neoplasms have been reported to associate with BHD syndrome, including three familial and one sporadic cases of renal tumors. We report another sporadic case with renal tumor. A 53-year-old woman complained of gross hematuria and visited our institute on November 1996. She visited the Department of Dermatology, Wakayama Medical College because of skin lesions on the face and upper trunk at her age of 44. These skin lesions were present since her mid twenties. Her daughter also had similar skin lesions and visited the same Department. There was no family history of renal tumor. The patient was diagnosed to have a right renal tumor, and radical nephrectomy was performed. Pathological diagnosis was renal cell carcinoma, papillary type. She underwent interferon injection therapy postoperatively, but died because of lung metastases on April 1997. This is the first reported case of renal tumor occurring in BHD syndrome in Japan.