Aromatic L‑amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive hereditary disease. Due to the lack of L‑amino acid decarboxylase, the synthesis and metabolism of monoamine neurotransmitters such as dopamine, catecholamine, and serotonin are impaired, resulting in autonomic nervous regulation disorders. Intraoperative hypotension, bradycardia, hypoglycemia, and body temperature fluctuations are significant concerns that present a great challenge for anesthesiologists. This article reports the anesthetic management of a 2‑year‑old boy with AADCD who underwent elective bilateral ligation of tunica vaginalis and left orchidopexy and discusses issues related to the perioperative management of children with AADCD to facilitate anesthesia management of patients who suffered this kind of disease.