We report a juvenile case of diffuse sclerosing osteomyelitis of the mandible that showed a favorable response to pamidronate, a bisphosphonate derivative. Although conventional treatments had been ineffective for 5 years, pamidronate administration brought about conspicuous improvement both clinically and radiographically. Severe adverse reaction was not found except for low-grade fever and lassitude on the day following administration. During the course of the treatment, however, nonsuppurative osteomyelitis of the right humerus also occurred, leading to the established diagnosis of chronic recurrent multifocal osteomyelitis. Pamidronate therapy was again performed successfully with near disappearance of clinical symptoms. Both bone-specific alkaline phosphatase (bone formation marker) and pyridinoline cross-linked carboxyterminal telopeptide of type I collagen (bone resorption marker) showed a marked decrease with pamidronate therapy, suggesting that pamidronate is useful for the treatment of chronic recurrent multifocal osteomyelitis with inhibitory effect on bone turnover.