Pilomatrix carcinoma, the malignant equivalent of pilomatrixoma, is rare among skin cancers. In the literature, there have been 80 cases of pilomatrix carcinoma reported, and among them nine were with metastases. The clinical presentation of this case is suggestive for the biology and of the usual history of this neoplasm. The patient was a 53-year-old male who had been treated 2 years earlier for a pilomatrix carcinoma located in the posterior part of the neck. The clinical presentation had been characterised by sudden paraplegia caused by vertebral collapse at T4 due to bone metastases. The patient underwent a first surgery for vertebral stabilisation and medullary decompression; then, he had a second operation for the resection of the local relapse of the tumour. Literature review and analysis of this case show that the pilomatrix carcinoma should be regarded as a highly locally aggressive tumour, with a high rate of local recurrence as well as metastases.