Rheumatic fever with renal involvement is rarely seen. The prevalence is found to be low although it may differ by reporters. We report a case of rheumatic fever with IgA nephropathy. The patient was a 14-year-old male. The patient's chief complaint were fatigue and gross hematuria. On the physical examination, the pansystolic murmur (grade II) on the left lower sternal border was noted. Cardiomegaly was seen on chest X-ray and pulmonary vasculatures were increased. Prolonged PR intervals were found on EKG. Echocardiogram showed rheumatic carditis with grade mild to moderate MR, regurgitant shunt from LV to LA through anterior leaflet. On the laboratory findings, ESR, CRP and ASO were increased, C3, C4 and IgA were normal. On the urine analysis, gross hematuria was noted as RBC over 30/HPF and RBC dysmorphism 90%. On the immunofluoroscent microscope of renal biopsy, IgA deposit on mesangial matrix was noted.