Neurofibromatosis type 1(NF-1) or von Recklinghausen disease is the most common of the neurocutaneous syndromes. It is characterized by pigmented cutaneous lesions and tumors of central nervous system. By the way, it could affect all the systems of the body. Sphenoid wing abnormalities are very rare and often associated with NF-1 so, they are considered pathognomonic. We report an unusual case NF-1 with unilateral absence of sphenoid wing and findings of multi-detector computed tomography and magnetic resonance imaging.