An 87-year old woman was admitted to emergency department troubled by several weeks of fatigue and a weeklong feeling of coldness in her fingers which were starting to blacken. Blood test revealed Hb 48g/l, reticulocytosis, high LD, low haptoglobin, DAT positive with C3d, +++, IgG+. Bone marrow aspirate flow-cytometry showed a small lambda B-cell clone not identical to B-CLL expressing CD20+, CD19+, CD5+, CD23+, CD10-. There were no signs of lymphoma on CT scans of neck, thorax and abdomen. Tests for virus or mycoplasma infection were negative. In the serum electrophoresis there was a M-component IgM, kappa, 12g/l. Repeated tests for cryoglobulins were negative. The diagnosis of Cold Agglutinin Disease was made. Treatment was commenced with prednisolone 40 mg and plasmapheresis and continued with a weekly rituximab and low-dose cyclophosphamide administered orally due to the high age of the patient. Laboratory signs of hemolysis diminished with time but never completely disappeared. In spite of the therapy, the finger necrosis progressed to 0,5cm and hand surgeons were contacted to follow the development of gangrene.