To evaluate the incidence and treatment results of osteosarcoma of the jaw (OSJ) in the Netherlands, data from 48 patients with a histologically proven diagnosis of osteogenic sarcoma of the maxilla or mandible were retrospectively analysed. Patient files, covering the period from 1964 to 1992, were obtained from all university hospitals in the Netherlands and the Netherlands Cancer Institute. The incidence of OSJ in the Netherlands is estimated to be at least 0.14 per 1,000,000. The overall 10-year survival was 59%. Distant metastasis occurred in 21% and local recurrences in 31% of the cases. Survival was significantly better in case of radical surgery and small tumours. Long-term survival after treatment of OSJ was good if complete surgical excision was achieved. Radiotherapy should only be considered to prevent local recurrence if surgery is not complete. The possible benefit of current chemotherapy in preventing metastatic disease is still questionable. Since other malignant neoplasms associated with OSJ occurred in 17% of the cases, lifelong follow up is mandatory for the detection of these second primary malignancies.