Pseudocholinesterase deficiency manifests as prolonged motor blockade after the administration of succinylcholine. A previously unknown homozygous form of the disease, became apparent during a lumbar laminectomy seriously limiting the ability to monitor motor evoked potentials and perform electromyelography (EMG). Moreover, concerns were raised as to how the enzyme deficiency would affect the metabolism of remifentanil and other esters during a total intravenous anesthetic. We present the perioperative management of the patient and a literature review of the syndrome. The patient provided written permission for the authors to publish this report. At our institution, IRB review and approval is not required for a single case report.