BACKGROUND: Malignant proliferating trichilemmal tumour is a rare skin tumour that originates from the root sheath of the hair and usually arises in the sun-exposed areas of elderly women. It mimicks poorly differential squamous cell carcinoma ant its biological behaviour is unpredictable, because rarely can produce distant metastases. CASE REPORT: The Authors report on a case of a 54-year-old female patients who proceeded with a cystic lesion in the scalp. The lesion was removed with wide excision. The histological examination of the lesion revealed a malignant proliferating trichilemmal tumour. Two years after the excison the patients is free of disease. DISCUSSION: Only few cases (about seventy) of malignant proliferating trichilemmal tumours have been reported in the Bibliography, in some of the patients with malignancy, the tumour produces distant metastases. In the reported case, although the high risk of distant metastasis, two years after the tumour resection the patient is free of disease, It is also reported a short review of the literature. CONCLUSIONS: Malignant proliferating trichilemmal tumours are rare malignant lesions that affect mainly older women. The appropriate treatment includes wide resection and dose postoperative follow-up of the patient to facilitate the early diagnosis of distant metastases.