Unfortunately, there is no definitive therapy available currently.<h4>Case report</h4>Here, we report a case of primary erythromelalgia and the treatment response in a 10-year-old boy, whose genetic findings for mutations in the SCN9A gene were positive and skin biopsy results were diagnosed as small fiber neuropathy, while he has suffered from excruciating burning pain, itching, erythema, and recurrent infections over the past 3 years. Here, SCN9A is linked to neuropathy, small fiber.