ABSTRACTIntroductionCongenital analgesia is a rare autosomal recessive hereditary disease. The primary damage of congenital analgesia is central structure damage of comprehensive pain perception.Case presentationA 1‐year‐old Han Chinese boy was admitted to hospital because of a tongue bite. He had no response to noxious stimulation of the body surface and was diagnosed with congenital analgesia. A small dose of remifentanil was intravenously injected during anesthetic induction to reduce the stress response caused by endotracheal intubation. A certain depth of anesthesia should be guaranteed during anesthetic induction and surgery to alleviate the stress response induced by endotracheal intubation and the operation.ConclusionOpioid analgesics are not required for general anesthesia in patient with congenital insensitivity to pain. With a heat dissipation barrier in patients with congenital insensitivity to pain with anhidrosis, body temperature, end‐tidal carbon dioxide and bispectral index should be monitored.