A 20-year-old primigravida with the autosomal recessive thrombocytopenia with absent radius (TAR) syndrome was followed in her third trimester of pregnancy. Although the platelet counts ranged from 8,000 to 30,000/microL, the patient had minimal symptoms of peripatellar ecchymoses. She presented at term with spontaneous rupture of the membranes and a double footling breech presentation. She successfully underwent low vertical cesarean section and delivery of a term, viable female infant. Management of the hemodynamic status was with blood component therapy. Antenatal ultrasonography demonstrated normal fetal extremities. Review of the literature revealed this to be the first report of cesarean section in a TAR syndrome patient. Management of coagulation problems should be individualized according to the severity of the disease.