<p>Atypical hemolytic uremic syndrome (aHUS) is a rare, chronic, and life-threatening thrombotic microangiopathy (TMA). The disease is most frequently linked to genetic or acquired dysregulation of the alternative complement pathway and affects both children and adults. The paucity of a gold standard for diagnostic testing and the variety of clinical manifestations are the primary reasons for the challenge in accurately diagnosing aHUS, which can have an impact on patient care. This is due to the fact that the more prevalent autoinflammatory diseases are multifactorial in nature, with both genetic and non-genetic factors playing critical roles. Until the last decade, treatment options were limited to plasma therapy and, in selected cases, liver transplantation, neither of which directly addressed the underlying pathophysiology and both of which carried substantial risks. Since its introduction in 2011, the anti-C5 monoclonal antibody eculizumab has significantly improved outcomes and transformed disease management. Trials on novel complement inhibitors, regenerative medicine, targeted therapy, and stem cell therapy may also improve future treatment options. The primary objectives of this investigation are to identify the genetic and epigenetic causes of aHUS, as well as the limitations and debates surrounding its management. Due to the prevalence and significance of this type of illness, researchers have investigated effective therapeutic methods and more recent approaches.</p>