Pilomatrixoma is a common benign adnexal tumour with differentiation towards matrix of the hair follicle. It is typically located in the head and neck region. The tumour is well circumscribed, lobular, cystic and composed of three major cell types: proliferating basaloid cells, eosinophilic squamous cells and shadow cells which represent dead cells. We report a rare case of malignant pilomatrixoma of the posterior occipital region in a 43-year-old female with metastases to C2 vertebra. The case was originally reported as proliferating pilomatrixoma. Bones of the head and neck region are a common site for metastases in malignant pilomatrixoma. Malignant pilomatrixoma is extremely rare. There are 90 cases regarded as acceptable reported in the English literature and ninety cases of metastasis. Because of its rarity, the precise incidence and prevalence of malignant pilomatrixoma are unknown. The diagnosis of malignancy is based on histological criteria which are not well defined and somewhat subjective. This includes cytological atypia, high mitotic activity with atypical mitosis, areas of confluent tumour necrosis, infiltrative growth pattern and vascular, lymphatic or perineural invasion.