Congenital insensitivity to pain with anhidrosis (CIPA, or hereditary sensory and autonomic neuropathy type IV) is a rare, autosomal recessive disease, is characterized by inability to sweat, insensitivity to pain, recurrent episodes of hyperpyrexia, self-mutilation and mental retardation. Because of lacking autonomic response to painful stimuli, it is difficult to determine adequate depth of anesthesia in patients with CIPA. We report an anesthetic experience for child with CIPA who had undergone an orthopedic operation. Anesthesia was induced by propofol and maintained by Sevoflurane with 50% nitrous oxide and anesthetic depth was monitored by using bispectral index (BIS). Throughout the operation, anesthesia was maintained with low end-tidal Sevoflurane concentrations (<1.5 vol%), BIS was within 28-62 and vital signs were stable. After operation, he did not remember anything about the surgery. The BIS monitor may be a useful tool to guide the adequate depth of anesthesia for the patient with CIPA.