Thrombocytopenia-absent radius (TAR) syndrome is a congenital malformation in which affected individuals present reductions in the number of platelets, hypoplasia, or absence of radial bone unilaterally or bilaterally. Hematologic, skeletal, cardiac (particularly tetralogy of Fallot and septal-atrial defects), and gastrointestinal anomalies are most commonly associated with TAR syndrome. Skeletal changes result in a higher risk of dental and craniofacial trauma in patients with the syndrome. Thus, it is important for the dentist to be aware of the characteristics of TAR syndrome and its clinical management for better care of these patients. The objective of this study is to describe a case report of a 26-year-old patient with TAR syndrome with a history of trauma and root fracture of tooth 11 and alveolar bone ridge. During anamnesis, root fractures requiring the extraction of the 11 tooth, alveolar bone ridge fracture in the adjacent region, and dental trauma were observed. A hematological evaluation and blood and radiological examinations were performed. Osseointegrated implant was performed using the guided surgery and flapless technique, as well as prosthetic rehabilitation in the affected region. This report discusses the importance of careful planning, such as the use of incisions and conservative surgery, techniques for alveolar ridge preservation, gingival manipulation, and prosthesis confection. The patient was attended by a hematologist throughout the treatment. Key words: TAR syndrome, absent radii and thrombocytopenia, dental implants, oral surgery.