Paget disease of bone (PDB) is the second most common metabolic bone disease. It is a chronic disease with a mono- or polyostotic appearance that is characterized by an increased bone turnover. The orthopedic surgeon is often confronted with such symptoms and complications as bone pain, skeletal deformities, and pathologic fractures caused by the \"out-of-balance bone remodeling process\". Careful evaluation of the clinical and radiographic findings is necessary to determine whether treatment of PDB is indicated. The mode of action of effective pharmacological treatments consists of reducing the increase in osteoclast-mediated bone resorption that characterizes the disease. Bisphosphonates are the compounds of choice for PDB therapy; these are readily available and have received approval. Patients with PDB are at increased risk for surgical complications, such as blood loss and heterotopic bone formation, if operative treatment is necessary. However, advances in surgical techniques and accompanying medical treatment could potentially improve the overall outcome of these patients. To achieve that goal, careful perioperative interdisciplinary management and monitoring are essential.