Oncogenic osteomalacia is an uncommon syndrome characterized by mineral metabolism abnormalities that disappear after the resection of an associated tumour. Head and neck is the second most frequent location of these tumours. We describe a case with an ethmoido-frontal phosphaturic mesenchymal tumour and review oncogenic osteomalacia-associated tumours. Among 21 cases found, 57 per cent affected the sinonasal area and 20 per cent the mandible. The diagnosis of the tumour lasted a mean of 4.7 years from the onset of osteomalacia, and most of them showed a significant vascular component. An aggressive surgical approach is recommended.