| Summary: | Plexiform neurofibroma (PNF) of the scalp is a very rare lesion. It is commonly associated with neurofibromatosis (NF 1). Occipital location of plexiform neurofibroma is even more infrequent. The usual presentation is an ill-defined mass causing disfigurement. It is evaluated with radiological imaging. Magnetic resonance imaging is preferred modality to identify the lesion and characterize its nature. It is usually managed by surgical excision, but recurrence has also been reported even after meticulous excision. The diagnosis is confirmed by histopathological examination of excised specimen. Here, we are reporting a case of plexiform neurofibroma with NF-1 inoccipital-cervical region with calvarial defect which was managed by surgical excision.
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