Plexiform neurofibroma is a presentation of neurofibromatosis type 1 (NF1) which can cause great facial deformities. Treatment rarely has a healing effect, so the surgical approach is aimed at improving esthetics and function. It requires a cross-disciplinary approach and typically needs multi-stage surgery. This is the case of a 16-year-old male patient with NF1 presenting with left periorbital and malar facial plexiform neurofibroma with slow-growth intraconal and extraconal invasion. He presented at the plastic surgery consultation for facial soft tissue deformity correction. Removal was performed using an esthetic subunit approach, with canthopexy and orbital cavity reconstruction, resulting in facial region symmetrization. This allowed for remarkable esthetic and functional improvement, facilitating ocular prosthesis adaptation. The subsequent use of selumetinib allowed the lesion to be stabilized.
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