A 62-year-old woman with medical history significant for chronic myeloid leukemia (CML) on maintenance nilotinib for the previous 5 years presented with a ten day history of descending weakness and subjective sensory loss, followed by dyspnea requiring emergent intubation. Neurologic exam revealed quadriparesis and areflexia with a seemingly intact though limited sensory exam. Initial MRI of the C spine was motion degraded but concerning for possible abnormality at C2. Lumbar puncture demonstrated albuminocytologic dissociation with protein 82mg/dL and 9 WBCs/uL. Nilotinib was discontinued due to case reports of association with acute inflammatory demyelinating polyneuropathy, and intravenous immunoglobulin therapy was initiated. Subsequent examination revealed a C2 sensory level with quadriplegia, and repeat MRI now demonstrated clear T2 signal abnormalities extending from C2-C7. MRI brain was without demyelination. Further negative work up included aquaporin-4 antibodies, myelin oligodendrocyte glycoprotein (MOG) antibodies, Mayo clinic cerebrospinal fluid paraneoplastic and autoimmune panels. The patient developed only minimal upper extremity improvement and required tracheostomy with subsequent transfer to a ventilator facility.