Fanconi anemia (FA) is a rare recessive DNA repair disorder that is clinically 
characterized by congenital malformations, progressive bone marrow failure, and 
increased incidence of malignancies, especially acute myeloid leukemia and 
squamous cell carcinomas of the head and neck (HNSCCs) and the anogenital 
regions. On a cellular level, typical features of the disorder are a high degree 
of genomic instability and an increased sensitivity to bi-functionally 
alkylating agents. So far, germ-line defects in 15 different FA genes have been 
identified. Some of these FA genes are also established as tumor susceptibility 
genes for familiar cancers.In recent years, the prevention and therapy of HNSCCs 
in FA patients has become more important as the percentage of patients surviving 
into adulthood is rising. HNSCCs appear in very young FA patients without common 
risk factors. Since cisplatin-based chemotherapy in combination with 
radiotherapy, essential parts of the standard treatment approach for sporadic 
HNSCCs, cannot be used in FA patients due to therapy-associated toxicities and 
mortalities even with reduced dosing, surgery is the most important treatment 
option for HNSCCs, in FA patients and requires an early and efficient detection 
of malignant lesions. So far, no uniform treatment protocol for the management 
of HNSCCs in FA patients exists. Therefore, we propose that the information on 
affected FA patients should be collected worldwide, practical therapeutic 
guidelines developed and national treatment centers established.