Neurofibromatosis-1 or von Recklinghausen's disease is an autosomal dominant disorder. Cafe au lait macules are generally the initial presenting feature of the disease, and there can be varying degrees of involvement of the skeletal, neurological, and pulmonary organ systems as the disease progresses.

We present a case report of disseminated fusariosis in a profoundly immunocompromised person. Early detection is key in this frequently-fatal infection; this case report emphasizes the findings with this infection and the need for chemoprophylaxis in appropriate patients.

Neurofibromatosis-1(NF-1) or von Recklinghausen's disease is an autosomal dominant disorder with an incidence of one in 2600 to 3000 people.[1] Cafe au lait macules are generally the initial presenting feature of the disease, and there can be varying degrees of skeletal, neurological, and pulmonary involvement as the disease progresses. The existence of Neurofibromatosis associated diffuse lung disease (NF-DLD) as a separate entity has been questioned since it was first reported in 1963 and, is often attributed to cigarette smoking, than a manifestation of NF-1.

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