Malignant proliferating trichilemmal tumour (MPTT) is a rare cutaneous tumour predominantly affecting the scalp, eyelids, neck and face of elderly women. It is a large, solitary, multilobulated lesion that may arise within a pilar cyst. These tumours are largely benign, often cystic and are characterized by trichilemmal keratinisation. However at times, the tumour has an aggressive clinical course and a propensity for distant metastasis. We report a case of a 29-year-old male who presented with a scalp swelling. Histology revealed malignant proliferating trichilemmal tumour of scalp. CT thorax showed multiple nodules in both lungs suggestive of lung metastasis. The patient underwent palliative radiotherapy and chemotherapy and expired 2 months after palliation. To the best of our knowledge, malignant proliferating trichilemmal tumour of the scalp with intracranial extension and lung metastasis is a rare condition, and only a handful of cases are reported till date. We report a case of malignant proliferating trichilemmal tumour of the scalp with intra-cranial extension and lung metastasis with relevant discussion at the same time.