Objective To explore the management strategies of ketone metabolic disorders. Methods Clinical manifestations, biochemical metabolic abnormalities, treatment and follow-up conditions of 3 children with metabolic crisis caused by ketone metabolism disorders were retrospectively analyzed. Of the 3 children, 2 had mitochondrial 3-hydroxy-3-methylglutaryl CoA synthase (HMGCS) deficiency, and 1 had succinyl-CoA: 3-oxoacid CoA transferase (SCOT) deficiency. Relevant literatures from CNKI, Wanfang, and PubMed databases before May 2025 were retrieved. Results The age of onset of the 3 children was 7 to 9 months old. The 2 children with HMGCS deficiency presented with severe metabolic acidosis accompanied by hypoglycemia, hepatomegaly, abdominal distension, hypophosphatemia, elevated blood acetylcarnitine/free carnitine and increased urinary dicarboxylic acid. After mechanical assisted ventilation and continuous renal replacement therapy in the acute phase, they improved. After stabilization, the main focus was on avoiding hunger, a low-fat and low-protein diet, and taking oral levocarnitine. Two children were detected with HMGCS2 gene compound heterozygous pathogenic variants, which were c.1498C>T and c.1502G>A, c.1465delA and c.520T>C respectively. All were known pathogenic variants. The two children had been followed up for 3 years and over 1 year respectively. There were no acute decompensated episodes. Their growth and development were normal. One child with SCOT deficiency presented with episodic ketosis, severe metabolic acidosis, with or without hypoglycemia and significantly elevated 3-hydroxybutyric acid in urine. In the past two years, there were five acute episodes. The acidosis was corrected within 24 to 48 hours. The child's growth and development were normal. A homozygous c.863delC variation of the OXCT1 gene of the patient was identified. No reports had been found. Conclusions Mitochondrial HMGCS deficiency and SCOT deficiency lead to severe ketone metabolic disorders and metabolic acidosis. It is necessary to identify the cause of the disease through early metabolic and genetic analysis. Metabolic intervention can effectively improve the metabolic condition.