✓ The authors present a histological study of resected tissue obtained from an 18-month-old girl with split cord malformation Type II whose right foot was smaller than her left. Magnetic resonance imaging revealed duplication of the spinal cord below the level of L-1. On laminectomy it was discovered that the cord was tethered in the lumbosacral region. The resected tissue contained the cluster of paramedian dorsal root ganglia unique to this congenital anomaly. On histological examination, the ganglion cells expressed not only neuronal markers but also a marker for muscle cells indicative of pathological development of the midline structure. Further histological study is necessary to gain a deeper insight into this congenital disease and to obtain additional information for use in surgical planning.