Diagnosis is divided into biochemical testing, which is dependent on the episodic or postprandial hyperammonemia, persistent hyperornithinemia, and urinary Homocitrullinuria, additional testing will show coagulopathy, and elevation in the liver enzymes [13]; while the genetic testing detects the mutations on the SLC25A15 gene [[10], [11], [12]]. Here, SLC25A15 is linked to Hyperammonemia.