Background <p>Split notochord syndrome is an extremely rare and controversial congenital anomaly considered part of the broader spectrum of complex spinal dysraphism and neurenteric developmental disorders. It results from abnormal embryological separation of the endoderm and ectoderm, leading to a range of vertebral, spinal cord, and potential enteric malformations. Due to significant overlap with other dysraphic conditions, diagnosis is primarily radiological and often challenging.</p> Case presentation <p>We report a case of a 10-day-old female neonate presenting with a dorsal lumbosacral swelling noted since birth, delivered at home to a mother with no antenatal care. Physical examination revealed a midline, skin-covered spinal mass without cerebrospinal fluid leakage. MRI of the spine, performed using a low-field (0.5 Tesla) system, demonstrated a midline vertebral defect, widening and segmentation abnormalities of posterior elements, and duplication of the spinal cord, with additional features suggestive of severe spinal dysraphism within the neurenteric spectrum. The diagnosis was based solely on imaging findings without surgical or histopathological confirmation. The constellation of findings was most consistent with probable split notochord syndrome within the neurenteric spectrum. The neonate received supportive management but deteriorated and died after seven days of hospitalization.</p> Conclusion <p>This case highlights a severe form of spinal dysraphism within the split notochord/neurenteric spectrum diagnosed on MRI in a resource-limited setting. It underscores the importance of early antenatal surveillance and postnatal imaging for prompt recognition of complex congenital spinal anomalies. MRI remains the key diagnostic modality for defining the extent of disease and guiding management, although overlap between entities within this spectrum may limit strict diagnostic classification.</p>