21 (3), 699-705 2006). This soft tissue neoplasm has gained an interest due to its diversity of histologic appearances, overlapping pattern, the rarity of the tumour, and the need of supportive and confirmatory tests which often renders MPNST as diagnostically challenging neoplasm. All these may justify to the delay in diagnosis. MPNST is rare, aggressive soft tissue sarcomas associated with poor prognosis. The diagnosis of MPNST relies on morphology and IHC interpretation. Differentiating MPNST from other types of spindle cell neoplasm is important for prognosis and management. Several markers have been suggested to distinguish MPNST from other soft tissue