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To present the clinical and imaging findings of two rare cases of bilateral orbital-cranio NF type I.

A retrospective study of clinical and imaging findings of two NF type I patients with bilateral orbital-cranio involvement.

A 33 yo man with extensive soft tissue infiltration involving the face and anterior-superior scalp and neck, ovoid mass posterior to the spinous process of C2, infiltration of the para pharyngeal spaces partially compressing the oropharynx, Bilateral orbital involvement with small calcified globe on the left side and sphenoid wing dysplasia, asymmetry of the skull vault with relative thinning on the left hemisphere.
A 15 yo female with generalized NF Type 1, severe bilateral plexiform neurofibromatous changes involving orbits, forehead and periorbital regions more marked on the right. Right sided extension in pterygopalatine fossa, infraorbital canal and foramen ovale.Plexiform neurofibroma left hemi-tongue, right sphenoid wing dysplasia, multiple foci of abnormal signal intensity involving right cerebellar hemisphere, brain stem globus palladi and thalami. Long term swallowing problems with high arched palate, small stature, longstanding cervicothoracic scoliosis.

Unilateral sphenoid wing dysplasia is one of the features of NF-1, but severe bilateral plexiform neurofibromatous changes involving orbits, forehead and periorbital regions are very rare.
Surgery remains the standard of care for these patients. Controversies remain about the timing of the first operation and today most multidisciplinary teams involving plastic, maxillofacial, ophthalmologic, and neurosurgeons favour early intervention to try to minimize the secondary deformation of the orbital and facial skeleton.