Hereditary sensory and autonomic neuropathy type IV is a rare disease characterized by fever episodes, mental retardation of different intensity, recurrent episodes of fever secondary to anhidrosis, little or no perspiration and congenital insensitivity to pain. Oral self-mutilation is also a characteristic sign. In this article, we present the case of an infant, aged 22 months, who showed these clinical characteristics and was treated with a dental device to prevent the patient from injuring her tongue. This device consisted of two acrylic splints joined at the back in the posterior sector, it provided an anterior open bite and allowed the infant to breathe through her mouth. The lesions of the patient had improved after using the device but the patient died due to the medical problem. Neuropathies treatment is a great challenge for medical teams.Dentists should form part ot these teams because of the bucal implications that may appear. Different appliances can be designed in order to solve the special problems each case may present.