α1-Antitrypsin (α1AT) deficiency is characterized by a high risk of developing pulmonary emphysema and liver disease. Arteriopathy may be associated with α1AT deficiency. We present a patient with only one kidney and α1AT 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 α1AT deficiency. After dialysis treatment for 15 months, the patient underwent an uneventful kidney transplantation. The case draws attention to the awareness of complications of α1AT deficiency even in cases with only mild pulmonary manifestations of the disorder. To our knowledge, atraumatic loss of a kidney in a patient with α1AT deficiency has not previously been reported.