A 48-year-old man, who was diagnosed as adult-type citrullinemia by quantitative estimation of men cycle enzymes in the liver, showed a regular nocturnal rice of blood ammonia level. In order to elucidate the mechanism of diurnal fluctuation of blood ammonia level, the patient was put into fasting state for four days. The blood ammonia level rose at the night of the first fasting day even though no food was taken, then it decreased gradually and reached the lowest level in the morning of the third fasting day. Intravenous administration of amino acids mixture under the same starved condition gave rise to a significant elevation of blood ammonia level. Based on these results, it was concluded that hyperammonemia of adult-type citrullinemia could result from the accumulation of free ammonia which was produced from thn catabolism of amino acids absorbed frcm the small intestine and surpassed the urea synthesis of defective urea cycle to flood into the blood. Furthermore, the rise of blood ammonia level at the night of the first fasting day suggested that the circadian rhythm of amino acid-carbohydrate metabolim might superimpcse on the process mentioned above, adult-type citrullinemia; hyperammonemia; diurnal fluctuation A 48-year-old man with adult-type citrullinemia has been described in our previous report (Yajima et al. 1981). He showed repetitive episodes of encephalopathy and was diagnosed by quantitative estimation of amino acids and urea cycle enzymes in the liver. In this case, a regular diurnal fluctuation of the blood ammonia level was seen and the drip infusion of glutamate-arginine mixture and oral administration of citrate lowered the blood ammonia level. But the liver function tests of the patient became worse one month after the laparotomy for the evaluation of urea cycle enzymes and he clinically deteriorated to the state of general liver failure with jaundice and ascites. He died two months after the laparotomy. At the stage of general liver failure, citrate ingestion was ineffective and regular diurnal fluctuation of blood ammonia was no longer seen.