Alpha1-Antitrypsin (alpha1AT) deficiency is characterized by a high risk of developing pulmonary emphysema and liver disease. Arteriopathy may be associated with alpha1AT deficiency. We present a patient with only one kidney and alpha1AT deficiency, but only mild pulmonary symptoms and no signs of liver disease, and atraumatic loss of the remaining kidney. Histological examination of the renal artery showed desposits of a mucoid ground substance in the arterial media, as has been demonstrated in other patients with alpha1AT deficiency. After dialysis treatment for 15 months, the patient underwent an uneventful kidney transplantation. The case draws attention to the awareness of complications of alpha1AT deficiency even in cases with only mild pulmonary manifestations of the disorder. To our knowledge, atraumatic loss of a kidney in a patient with alpha1AT deficiency has not previously been reported.