Congenital insensitivity to pain with anhidrosis (CIPA) is a rare hereditary sensory and autonomic neuropathy. CIPA is characterized by inability to sweat, insensitivity to pain, self-mutilation, recurrent episodes of hyperpyrexia, mental retardation, and autonomic nervous system abnormality. Patients with CIPA may undergo surgery because of susceptibility to trauma, bony fracture and osteomyelitis due to insensitivity to pain. We report a child who had undergone anesthesia with total intravenous anesthesia. The anesthetic management of this condition is discussed.