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Fanconi's disease (FA) is a rare genetic disease with autosomal recessive transmission; which combines the classic triad, short stature, malformation syndrome, bone marrow failure. The main malformations concern the extremities, the spine, the skin, the kidneys, the urinary tract and the otolaryngological sphere. Advances in molecular biology have enabled the identification of 15 genes involved, whose mutation is responsible for hematological damage and increased neoplastic risk. Patient care is multidisciplinary, it is hematological and extra-hematological, associating screening for organ deformities and deficits with monitoring for the appearance of precancerous or tumor lesions. We report a case of a 41-year-old patient with squamous cell carcinoma of the lower lip, classified T3 N0 M0 and whose preoperative assessment awakened Fanconi's disease. The patient was operated on requiring surgical excision for carcinological purposes with immediate repair of the loss of substances (LOS).
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