Fanconi's anemia (FA) is an autosomal recessive disorder characterized by constitutional aplastic anemia and congenital abnormalities. Patients with this disorder are prone to develop leukemia. Besides the risk of squamous cell carcinoma (SCC), development especially in the head and neck region is also increased. Up to now 40 patients with FA have been reported to develop SCC, and in 14 of them the tongue was the primary site. All of the reported SCC in FA patients originated in mucosal and mucocutaneous sites, especially oral (n=25) and anogenital sites (n=8) and the esophagus (n=6), with the exception of two patients with multiple cutaneous involvement. We report a new case of SCC of the tongue in a patient with FA and review the previous SCC cases.