ABSTRACT
Fanconi anaemia (FA) is a rare autosomal recessive inherited disease, which consists of bone marrow failure, skeletal deformities and multi organ nvolvement. Here, we report an emergency surgery on a FA patient with mucormycosis infection of paranasal sinuses. Since there are a few reports about FA, there is no general consensus on anaesthetic considerations so we deal with recommendations that are based on the pathology of FA. Possible difficult air way is the main concern, and low oxygen concentration and avoidance of nitrous oxide are among recommendations.