Split cord malformations (SCMs) are thought to be rare abnormalities representing 3.8-5% of all spinal cord anomalies. The prevalence is estimated to be 1 in 5499 live births (0.02%), with a slight female predominance (1.3:1). Although the estimates of prevalence vary, Type I SCM occurs more frequently than Type II SCM. In this paper, we are reporting the clinical presentation and imaging findings of multi-level SCM in a 27-year-old male. A literature review of the embryological background of SCM and pathological hypothesis for this entity is provided. A systematic review has been conducted to identify multi-level SCM cases reported in the literature, followed by proposing a new classification system to further our understanding and management of SCMs.