A previously healthy 19-year-old girl was admitted to our hospital because of hyperkalemia. Pseudohyperkalemia was diagnosed because there was a marked difference between levels of serum and plasma potassium. Her plasma potassium level was markedly increased after 6-hour in vitro incubation of blood at room temperature, suggesting excessive potassium release from red blood cells without coagulation. The plasma potassium levels of the patient and her father were markedly elevated in blood specimens incubated in vitro at 4 degrees C, but not at 37 degrees C. These data indicated pseudohyperkalemia syndrome caused by abnormal leakage of potassium from red blood cells at the lower temperatures.