Although data from several studies support the use of arginine vasopressin (AVP) for the treatment of hypotension concomitant with pulmonary hypertension (PH) in the cardiac surgery setting, to our knowledge, no previous studies have reported the effect of AVP on the systemic and pulmonary circulation of patients with PH secondary to lung diseases. In this report, we present the hemodynamic responses to bolus administrations of AVP and noradrenaline in a patient with PH secondary to pulmonary emphysema. The patient showed low systemic vascular resistance hypotension during off-pump single-lung transplantation. The bolus administration of AVP (0.5 U) increased systemic arterial pressure by 35.2%, with a minimal change in pulmonary arterial pressure, resulting in a significant decrease in the pulmonary arterial pressure/systemic arterial pressure ratio. In contrast, the bolus administration of noradrenaline (10 or 20 μg) increased both systemic and pulmonary arterial pressures by 14.8 and 6.7%, respectively. In summary, the bolus administration of AVP effectively increased systemic arterial pressure with a minimal effect on pulmonary arterial pressure in a patient with PH secondary to pulmonary emphysema. This case highlights the potential utility of AVP to treat low systemic vascular resistance hypotension in patients with PH secondary to lung diseases.