Background: Copper deficiency leads to hematologic disorders like pancytopenia. In addition, myeloneuropathy was described in a few cases reports. Case Report: A 71-year-old woman was hospitalized because of increasing pancytopenia and ataxic gait, that resulted in a near-complete inability to walk without assistance. Additional symptoms included arthralgia, reduced appetite and weight loss. Laboratory studies revealed a proteinuria of 3,700 mg/day. Magnetic resonance imaging of the cervical and thoracic spine revealed a wedge-shaped signal intensity in the dorsal part as a sign of damage in this area. A copper deficiency was then identified as the likely underlying cause for the low blood cell counts and neurologic deficits. In this patient, the copper deficiency may have resulted from a disturbance in absorption due to a partial gastrectomy (modified Billroth 1) 10 years ago and due to urinary copper loss in view of mesangioproliferative glomerulonephritis. A therapy with copper gluconate 3 x 3 mg/day was initiated. Within 2 weeks, blood cell counts normalized and appetite became normal again; just so, arthralgia disappeared. The neurologic symptoms persisted, even though the copper substitution continued for 6 months. Conclusion: Copper deficiency may be a differential diagnosis for hematologic abnormalities like pancytopenia, even if a disorder of intestinal resorption or a proteinuria occurs. Myeloneuropathy is a rare complication of this deficiency. Hemograms may become normal after treatment with oral copper gluconate, but at least in the case presented here, neurologic symptomes did not show any improvement.