INTRODUCTION: Limited dorsal myeloschisis (LDM) is a rare form of spinal dysraphism characterized by two constant features: a focal ”closed” midline skin defect and a fibro neural tract connecting the skin lesion to the underlying spinal cord. Excision of the tract followed by reconstruction is the preferred management. Intraoperative neurophysiologic monitoring (IONM) is widely used in neurosurgery to recognize important neurological structures but can be challenging in the pediatric population due to incomplete neural development.

CASE REPORT: A 7-day-old male baby presented with a history of dorsal ”cigarette burn” skin lesion noted at birth. Clinical assessment and imaging findings revealed a fibro neural tract extending from the skin surface into the lumbar spinal canal at the level of L5-S1. Excision of skin lesion/ tract and untethering of filum terminale with dural tube reconstruction under Intraoperative neurophysiological monitoring (INOM) was performed. The procedure went smoothly. Postoperatively, the child showed no neurological compromise or dermatological complications. In addition, the wound healed well, and the neonate was completely normal.

CONCLUSION: This case demonstrates the successful management of LDM through an IONM-guided complex reconstruction surgery in a neonate and the effective use of IONM in such challenging cases.