Malignant peripheral nerve sheath tumour (MPNST) is a rare soft tissue sarcoma with high rate of local recurrences and metastases. Common sites include deeper soft tissues, usually in the proximity of a nerve trunk. MPNST of breast is a rare occurrence. To our knowledge there are only less than 10 case reports of MPNST of breast. We present a young male patient with MPNST and lymph node metastases and also discuss diagnostic modes and management challenges in such rare difficult scenarios.