A 2 year old, castrated male West Highland White Terrier was referred for evaluation of vomiting, anorexia, polyuria, polydipsia, and lethargy. Copper storage disease and fanconi's syndrome was diagnosed via quantitative liver copper analysis, copper stains, and routine histological evaluation of a laparoscopic liver sample. Fanconi's syndrome was diagnosed based on an increased fractional excretion of amino acids, organic acids, and electrolytes, and glucose in the face of euglycemia. The dog received supportive therapy and was treated with D-penicillamine, ursodiol, vitamin E, S-adenosylmethionine. At four-month recheck he was clinically normal and the fanconi's syndrome has resolved.