The authors report an unusual case of hemilaryngopharyngeal spasm (HeLPS) with glossopharyngeal neuralgia with otalgia in a middle-aged female patient. Her symptoms included left-sided sharp, electric sensation like pain radiating from her left ear to the left side of her throat. The intermittent symptoms were worse with swallowing, laughing, talking, hoarseness of voice and activities associated with increased heartrate. Her symptoms were severe enough to cause vomiting and bladder incontinence. Trials with Carbamazepine, and Gabapentin failed to improve her symptoms. Neurological exam was normal between symptoms. A magnetic resonance imaging (MRI) study showed her left posterior inferior cerebellar artery (PICA) curving into and posteriorly distorting her left lower cranial nerves. Patients symptoms resolved after microvascular decompression of a loop of the posterior inferior cerebellar artery which was compressing the IXth and Xth cranial nerves. The authors describe 27 patients from published worldwide literature with symptoms similar to HeLPS. The condition sounds so similar to hemifacial spasm but with neurovascular compression of the IXth and Xth nerve instead of the VIIth. Otolaryngologists could consider this differential diagnosis in patients presenting with similar symptoms.