Congenital insensitivity to pain is a rare childhood disorder. Five different types of hereditary sensory and autonomic neuropathy have been identified to date, each one with diverse clinical features. We report the clinical case of a 10-year-old boy, diagnosed with hereditary sensory and autonomic neuropathy type IV, an autosomal recessive condition. This disease is characterized by insensitivity to pain and temperature, anhidrosis, self-mutilation and mental retardation. Anhidrosis leads to thermoregulation disorders, which might cause episodes of fever and insensitivity to pain associated with recurring fractures and joint damage.

autonomic neuropathy; anhidrosis; self-mutilation; insensitivity to pain