Diffuse sclerosing osteomyelitis is an uncommon and poorly understood chronic inflammatory disease, for which many etiological theories and treatment modalities have been suggested. Recurrent swelling and pain in the lower jaw are typical symptoms, often accompanied by trismus. The management of diffuse sclerosing osteomyelitis is a challenging and often long term with several drug therapies being reported in the literature, including anti-resorptive therapies. This study aimed to investigate the effects of bisphosphonates therapy in diffuse sclerosing osteomyelitis patients and report an atypical case in a young patient. A systematic review of the literature was performed including 130 patients with diffuse sclerosing osteomyelitis treated with bisphosphonates. Complete resolution of the clinical symptoms, with regression of pain and swelling were reported in 105 (80,76 %) patients. Pamidronate IV was the most frequent medication applied with stable and satisfactory long-term results. In this study, the patient was treated with intravenous administration of 45 mg of pamidronate disodium, once a day, for three continues days. The follow-up was uneventful with complete regression of the clinical symptoms. Bisphosphonates seem to be a good option in the treatment of diffuse sclerosing osteomyelitis in the jaw, especially pamidronate IV.