Perioperative management of orthopaedic patients with a hematologic disorder is a complex endeavor that requires a multidisciplinary team-based approach. A team composed of an experienced orthopaedic surgeon, an anesthesiologist, and a hematologist is necessary to achieve optimal outcomes. Patients with hemophilia and other complex hematologic disorders should be managed at, or in consultation with a hematologist at, a comprehensive hemophilia center. Bleeding disorders and inherited thrombophilia present unique challenges for the perioperative management of orthopaedic surgery. Comprehensive preoperative planning and familiarity with treatment guidelines can help to minimize these risks. Knowledge of the disease processes outlined in this article will provide orthopaedic surgeons with the requisite background knowledge that is needed to initiate safe and effective treatment strategies involving this high-risk patient population.