Benign epithelioid peripheral nerve sheath tumor, a rare entity is an umbrella term describing benign, neural origin tumors with epithelioid morphology. Clinically indistinguishable from other benign cutaneous lesions, histopathology offers the only source of accurate diagnosis. Morphologic mimics include many benign and malignant soft tissue lesions. Besides a predominant epithelioid component, the lesion can also show a fair share of spindle cells. A circumscribed nodular tumour of low mitotic activity, it often exhibits areas resembling schwannoma or neurofibroma. An awareness of this entity and its varied morphological aspects helps to arrive at the correct diagnosis and hence avoid unnecessary extensive surgical procedures. This case presents features of this benign tumor which occurred in a 47 years old man.
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