We studied a 17-year-old girl with exercise-induced anaphylaxis (EIA). She experienced 10 episodes of EIA which demonstrated cutaneous, digestive and cardiorespiratory symptoms. This subject exhibited elevation in plasma histamine (13.3 ng/ml) and decrease in CH50 (17 U/ml) and C3 (70 mg/dl) after naturally induced EIA. On remission day CH50 (26 U/ml), C3 (83 mg/dl) and C4 (15 mg/dl) were of relatively low values. Her mother demonstrated the same tendency with a low level of C4 (14 mg/dl), and this suggested an underlying genetic disorder in the complement system. Exercise challenge could not elicit EIA symptomatically but showed fluctuation of CH50. The triggering mechanism of EIA was unclear; however there is a possibility that complement plays an important role in EIA.