This report describes a rare tumour, an epitheloid haemangioendothelioma affecting the lower lip. This tumour has a predilection for the head and neck region in young adults. Its potential to metastasise is well recognised, but the likelihood of this is currently uncertain. Current management is usually to locally excise the tumour and follow up, although there is a recognised risk that subsequent presentation with metastatic nodal disease can occur. We present a case occurring in the lip of an 18-year-old girl who had a sentinel node biopsy performed as a staging tool in conjunction with excision of a local recurrence. Although clinical examination and CT imaging of the head and neck found no evidence of metastatic disease, the sentinel node was found to contain metastatic tumour. The result of this unexpected finding was that she was investigated further with additional CT scanning of her chest and abdomen. Subsequently, a therapeutic modified radical neck dissection preserving the accessory cranial nerve was undertaken. After 3 years she remains well with no evidence of recurrent tumour. We believe that the consequence of undergoing sentinel node biopsy, which detected early metastatic tumour and her subsequent treatment, suggests a role for sentinel node biopsy in the management of epitheloid haemangioendothelioma.