Background: Occipital neuralgia associated with migraine or cervicogenic headache is not uncommon clinical syndrome. It’s still a debatable issue regarding the pathogenesis and treatment options in the literature. Objective: It was to study and analyze the rule of microsurgical decompression of C2 nerve and it’s ganglion in management of intractable occipital neuralgia with migraine. Patients and Methods: Thirty-six patients with this syndrome had been subjected to full clinical assessment to fulfill the clinical criteria of having occipital neuralgia with migraine (cervicogenic headache). All patients were subjected to image guided C2 ganglion anesthetic block and corticosteroid administration as a therapeutic test. Results: Among the 10 patients who had surgery and during a mean full up period of 28.1 months, 6 patients 60% (7 sides) were totally free of pain. Three patients (30%) showed moderate degree of improvement with less frequent attacks and controlled with minor medication. Only one patient (10%) showed no improvement but still without worsening of his preoperative symptoms. In nine patients (90%) the main pathology was vascular compression by vertebral venous plexus around the root and ganglion, while in six (60%) patients we found the hypertrophied dorsal atlanto-epistrophic ligament is the main pathology. Osteoarthritic sharp lower border of C1 vertebrae and lateral mass were found in three patients (30%). Conclusion: Good selection of patients with typical clinical presentation together with C2 ganglion anesthetic block were the key of success of microsurgical decompression of C2 nerve and ganglion as a valid treatment option in intractable occipital neuralgia with migraine.