Objective: we describe a case of a patient with familial dysautonomia and postoperative pain. Methodology: clinical follow-up for 10 years in a tertiary pediatric hospital. Results: it is a 12 years old teenager, diagnosed with Riley-Day Syndrome, after undergoing various orthopedic surgical procedures, evolves with changes in pain perception, in systematic assessments. Conclusion: Family Disautonomy is one of the most common Hereditary Sensory and Autonomic Neuropathies (HSAN). Children gradually evolve with sensory changes, leading to a progressive decrease in pain perception. In addition, spinal deformities and recurrent orthopedic trauma can occur. The reported patient presents, contradictorily, an increase in the perception of pain and allodynia after undergoing repetitive orthopedic procedures.
