Background: The erector spinae plane (ESP) block is increasingly used for pediatric thoracic analgesia, yet evidence in infants under one year remains limited. Thoracotomy is associated with severe postoperative pain and high opioid requirements. Autosomal recessive congenital ichthyosis (ARCI), characterized by impaired epidermal barrier function, raises concerns regarding the safety of regional anesthesia. No previous report has described the use of regional anesthesia in patients with ARCI.Case: A 10-month-old, 9.2 kg male infant with genetically confirmed ARCI (PNPLA1 mutation) underwent left posterolateral thoracotomy and decortication for loculated empyema. A pre-incision ultrasound-guided ESP block was performed at T4 using 0.25% bupivacaine (4.5 mL; 1.22 mg/kg). Intraoperative opioid use was limited to fentanyl 25 mcg for intubation, with no additional analgesic requirement or hemodynamic response to surgical incision. Postoperative pain scores assessed using the face, legs, activity, cry, consolability (FLACC) scale remained 1/10 during the first 24 hours, increased to 6/10 on post-operative day (POD)-1, and subsequently decreased to 4/10 on POD-2 and 3/10 on POD-3 under multimodal non-opioid analgesia. No rescue opioids or opioid-related adverse effects occurred. The patient was extubated in the operating room and discharged from the pediatric intensive care unit (PICU) after 58 hours. No block-related complications were observed, and puncture-site healing was uneventful.Discussion: This case demonstrates that the ESP block provided effective opioid-sparing analgesia in an infant and suggests that ARCI-related epidermal barrier dysfunction did not appear to adversely affect the safety or technical performance of an interfascial plane block in this case.Conclusion: ESP block appeared to be a feasible, effective, and safe analgesic option for thoracotomy in this infant with ARCI and may serve as an opioid-sparing strategy in high-risk pediatric patients.