Acute poststreptococcal glomerulonephritis (APSGN) is a well-established disease. Although various immune responses are thought to be involved in the pathogenesis of APSGN, the disease has a self-limiting nature in clinical practice, despite the presence of severe acute symptoms. We herein report the case of a 78-year-old woman with APSGN who developed primary biliary cirrhosis (PBC) after achieving remission of renal manifestations, including anasarca and elevation of serum creatinine, indicating that persistent alterations in the immune system can cause extrarenal disorders. This case provides insights into the appropriate clinical management of ASPGN and pathogenesis of PBC.