Vascular compression is a well-established cause of cranial nerve neuralgic syndromes. A unique case is presented that demonstrates that vascular compression may be a possible cause of occipital neuralgia. A 48-year-old woman with refractory left occipital neuralgia revealed on magnetic resonance imaging and computed tomographic imaging of the upper cervical spine an atypically low loop of the left posterior inferior cerebellar artery (PICA), clearly indenting the dorsal upper cervical roots. During surgery, the PICA loop was interdigitated with the C1 and C2 dorsal roots. Microvascular decompression alone has never been described for occipital neuralgia, despite the strong clinical correlation in this case. Therefore, both sectioning the dorsal roots of C2 and microvascular decompression of the PICA loop were performed. Postoperatively, the patient experienced complete cure of her neuralgia. Vascular compression as a cause of refractory occipital neuralgia should be considered when assessing surgical options.