Osteomyelitis is an inflammatory process involving cortical and cancellous bone. In the maxillofacial region, the mandible is the most frequently affected bone. In the vast majority, a bacterial focus can be identified as the origin of the disease. Chronic progress of the disease may lead to destruction of mandibular bony structures, resulting in mild or severe loss of function if no adequate treatment is applied. In some cases, the etiology of osteomyelitis remains unclear. Review of literature revealed two cases of necrosis of the mandibular condyle caused by primary osteomyelitis. We report a case of primary osteomyelitis of the mandibular condyle in a 51-year-old woman. Radiography revealed an almost complete destruction of the right mandibular condyle, resulting in malocclusion. The patient was treated with long-term antibiotics. No surgical intervention had been performed. After remission of the symptoms, the malocclusion had been corrected prosthetically. After a 4-year follow up period, the occlusion is stabile and there are no signs of progression of the disease.