Trichoepitheliomas are benign epidermal appendage tumours that present most commonly as solitary lesions, and less often as multiple, symmetrically distributed lesions on the face, scalp, neck and trunk. Only a few patients have been reported in whom trichoepitheliomas were found as unusually configured linear or plaque-like confluent lesions, all of which occurred in patients with skin types V or VI. We describe a white girl with a hemifacial plaque of confluent naevoid trichoepitheliomas, the first report of such lesions in a white patient.