The presence of two male cases with MPNST aged 32 (retro-orbital) and 33 years (lower limb) and one female case of triton tumour aged 5 years amongst carriers of a 524 G > A (Arg175His), a 574delC and a 375 G > A TP53 mutation respectively suggests a likely association with Li Fraumeni syndrome. Here, TP53 is linked to Li-Fraumeni syndrome.