The authors describe the case of a 36-year-old woman with bilateral internal jugular vein occlusion, hydrocephalus, and Dandy-Walker variant who presented with myelopathy that was ultimately attributed to ventriculoperitoneal (VP) shunt failure. Computed tomography (CT) angiography of the head and neck revealed epidural venous engorgement within the cervical spine, greater that 50% narrowing of the C2-5 spinal canal, and compression of the cervical spinal cord. After successful shunt revision, postoperative CT angiography revealed decreased venous engorgement as well as decompression of the cervical spinal cord, and the patient's myelopathy improved. This case represents a fascinating clinical presentation of VP shunt failure, highlighting the physiological importance of the external jugular pathways involved in cerebral venous drainage.