Though the role of Blood Brain Barrier (BBB) is well studied in brain disorders, its functional equivalent, the blood-spinal cord barrier (BSCB), lacks in-depth investigation regarding its role in spinal cord and neurological disorders as it is still considered simply an extension of the BBB. The major structural constituents of both the BBB and BSCB are similar, yet functional differences in their role in diseases of the spinal cord exist. Compared to the brain, the spinal cord is a far more agile organ that is surrounded by supporting diverse tissues and extracellular matrices. In the event of dysfunction, immune responses generated from the spinal cord clinically differ from those in the brain and require a suitable intervention. Dysfunction of the BSCB is associated with various traumatic and non-traumatic injuries, aging and cancer and should be considered as a separate entity for research and clinical purposes. Thus, it is crucial to understand the composition of BSCB and also to establish the cause-effect relationship with aberrations and spinal cord dysfunctions. We have done a descriptive analysis of the anatomy, current techniques to assess the impairment of BSCB, associated risk factors and impact of spinal disorders such as spinal cord injury (SCI), amyotrophic lateral sclerosis (ALS), peripheral nerve injury (PNI), ischemia reperfusion injury (IRI), degenerative cervical myelopathy (DCM), multiple sclerosis (MS), spinal cavernous malformations (SCM) and cancer on BSCB dysfunction. Along with diagnostic and mechanistic analyses, we also provide an up-to-date account of available therapeutic options for BSCB repair. We emphasize the need to address BSCB as an individual entity and direct future research towards it.