Autoinflammatory bone disorders are characterised by dysregulation of the innate immune system resulting in episodes of sterile bone inflammation. They may be associated with inflammation of other organ systems such as the skin and intestines. Both sporadic and monogenic variants have been described. Although the precise pathogenesis of chronic non-bacterial osteomyelitis (CNO) is not well understood, mouse models have helped further our understanding of this disease. In the absence of validated criteria, clinical tools and criteria that can aid in diagnosis have been proposed. Whole-body magnetic resonance imaging (WB-MRI) is widely used for diagnosis and monitoring of disease activity. In select cases, bone biopsy may be required to exclude other conditions. Treatment practices vary between regions but non-steroidal anti-inflammatories (NSAIDs), bisphosphonates and biologics are usually preferred.