INTRODUCTION: Situs inversus is a rare condition of visceral transposition in which the spinal axis is rarely affected. CASE REPORT: The authors report a patient with situs inversus totalis and type II split cord malformation. This patient had no complaints and presented with scoliosis. CONCLUSIONS: Recent compelling evidence from animal models and human case reports has led to hypotheses that defects of the midline and laterality defects (e.g., situs inversus) are etiologically related. Confirmation from additional case reports of situs inversus and split cord malformation could prove useful in determining a genetic locus for split cord malformations or implicating various chemical agents that are known to produce situs inversus as potential causative factors in the production of split cord malformations.