Peripheral nerve sheath tumours [neurofibroma, malignant peripheral nerve sheath tumours (MPNST)] and schwannoma arise from the Schwann cell lineage and can be genetically characterised as Neurofibromas (either dermal or plexiform) and MPNST’s [Neurofibromatosis 1 (NF1)], or Schwannomas [Neurofibromatosis 2 (NF2)], Schwannomatosis and Carney complex type 1. Here, NF1 is linked to plexiform neurofibroma.