Vanishing bone disease is a very rare, peculiar destructive condition of the skeletal system resulting in spontaneous and progressive resorption and disappearance of osseous structures with replacement by vascular, fibrous connective tissue. Children and adolescents are most commonly affected, although patients ranging in age from 18 months to 40 years have been reported. Its clinical presentation is highly variable largely depending upon the site of skeletal involvement. The maxillofacial territory and upper extremity bones are the predominant sites affected by this ailment. The etio-pathogenesis of this condition still remains poorly understood, the natural history and prognosis is unpredictable. Radiographic findings associated with vanishing bone disorder are particularly dramatic and characterized by complete resorption and disappearance of the affected bone leading to the definition of “vanishing bone” or “disappearing bone disease.” Although various treatment options have been suggested, in general, no single treatment modality has proven effective in arresting this rare clinical entity. The present article provides an insight into the in-depth comprehensive review of literature pertaining to this fascinating musculoskeletal disorder in children.