The renal defects resulting in a Fanconi syndrome were seen in eight Basenji dogs by measuring renal clearance and in vitro amino acid and sugar uptake and performing histopathologic evaluations. Renal tubular handling of glucose, phosphate, sodium, potassium, uric acid, and amino acids was abnormal, and in vitro uptake of labeled lysine, glycine, and alpha-methyl-D-glucoside by renal cortical slices was impaired. Histopathology was normal except for enlarged nuclei in some renal tubule cells. These Basenji dogs, which may be genetically affected, represent a likely model for idiopathic Fanconi syndrome in humans.