Previous studies demonstrated the significance of an agonistic angiotensin II receptor AT1 autoantibody (AT1-AA) in preeclampsia. Because of its ability to release calcium in vascular smooth muscle cells, stimulate reactive oxygen species, and initiate proinflammatory processes, this antibody was thought to be important in the etiology and pathogenesis of preeclampsia. Recent investigations, however, have broadened and refined the pathobiological relevance of this antibody and refuted its role as the primary cause for preeclampsia. Because AT1-AA has been linked to an impaired uteroplacental perfusion and has been detected in patients with renal allograft rejection, its occurrence and function seem to be wider and more complex. This review summarizes current knowledge about the generation, function, and clinical importance of AT1-AA.