OBJECTIVE AND IMPORTANCE: Medically refractory occipital neuralgia (ON) has been treated with a variety of neuroablative procedures. The present case report supports the effectiveness of ventrolateral partial rhizotomy (pVL-DREZ) of the C1 to C3 cervical dorsal roots, a relatively unknown procedure.CLINICAL PRESENTATION: A 46-year-old woman had a 14-month history of severe right-sided ON. Multiple trials of medical treatments, nerve blocks and local steroid injections had failed. Her daily opioid requirements had escalated to include frequent injections in addition to prescribed oral opiates.INTERVENTION AND RESULTS: A pVL-DREZ at the right C1 to C3 level was performed through a C1 laminectomy and C2 right laminotomy. The ipsilateral upper cervical dorsal roots were exposed and a 1 mm deep incision was made at a 45° angle to the sagittal midline at the ventrolateral aspect of each dorsal rootlet entry. The patient experienced postoperative opioid withdrawal seizure and transient disequilibrium for two weeks. Touch sensation was preserved and complete abolition of ON over a four year follow-up was achieved.CONCLUSION: The pVL-DREZ procedure provided complete pain relief for the patient and avoided the potential complications often encountered with other destructive interventions. pVL-DREZ should be considered among the available options for the treatment of refractory ON.