Carnitine palmitoyltransferase II deficiency is an inherited disorder affecting mitochondrial long-chain fatty acid oxidation. The severe infantile form typically presents between 6 and 24 months with recurrent hypoketotic hypoglycemia, seizures, liver failure, and transient hepatomegaly. Heart involvement with cardiomyopathy and arrhythmia can also occur. Episodes are triggered by infections, fever, or fasting. Laboratory studies show hyperammonemia, metabolic acidosis, and hypoketotic hypoglycemia with elevated creatine kinase levels. There are three forms of the disorder: lethal neonatal, severe infantile, and myopathic, all caused by mutations in the CPT2 gene. The neonatal form leads to respiratory and liver failure, cardiomyopathy, and hypoketotic hypoglycemia. The severe infantile form involves liver, heart, and muscle issues, with risks of liver failure, nervous system damage, and sudden death. The myopathic form is characterized by muscle pain and weakness, with episodes triggered by various factors. Males are more commonly affected than females.