Melorheostosis is a rare condition of bone and surrounding tissues with different clinical presentations, ranging from clinically inapparent radiographic incidental findings to deformity, resistant chronic pain, swelling, restricted range of motion of the affected joints, leg length discrepancy or even cervical myelopathy. It is characterized by a hyperostosis of long or short bones. It is often associated with cutaneous and vascular changes like naevi, haemangiomas, scleroderma, lymphedema and arteriovenous malformations. Pain is the most common symptom of the condition. The therapy is individual and depends on the clinical manifestation. There are about 350 cases described in the literature until today. The current article presents an actual and most comprehensive review of literature to this rare condition, including 313 cases, focusing on symptomatology, diagnostic steps and the therapeutic options.