This report describes a case of transient hyperammonemia following tonic-clonic status epilepticus with an initial ammonia level of 537 Umol/L. This appears to be the highest transient ammonia level reported in the literature in this clinical scenario. This is an affirmation that an initial elevated ammonia level in the absence of hepatic dysfunction should be interpreted with caution when associated with status epilepticus. Repeat levels should be drawn to identify transient hyperammonemia and determine the need for treatment if levels do not decrease.