Abstract

Fanconi's anemia (FA) is a rare autosomal recessive syndrome associated with a strong predisposition to cancer, particularly squamous cell carcinoma (SCC) of various organs. A few cases of lower genital tract neoplasia have been described. We present a 14-year-old black girl with an advanced squamous cell vulvar carcinoma treated with cisplatin chemotherapy plus radiation therapy. The patient died because of fungal sepsis. Polymerase chain reaction (PCR) was positive to human papillomavirus (HPV)-16. Vulvar carcinoma is a very rare condition in teenagers, but the association of Fanconi's anemia and SCC of many sites is common. Vulvar carcinoma when associated with Fanconi's anemia is a great treatment challenge.