Although palatal myoclonus has been extensively described (Deuschl, Mischke, Schenck, Schulte-Mönting, & Lücking, 1990), little is known about pharyngo-laryngo-palatal myoclonus (PLPM), a complex and rare subset of head and neck myoclonic disease. To date, there have been only 5 cases of PLPM reported (Drysdale, Ansell, & Adeley, 1993; Gupta, Samant, & Katiyar, 1972; Ito, Kimura, & Shibasaki, 1993; Juby, Shandro, & Emery, 2014; Toland, Porubsky, Coker, & Adams, 1984), and no review of the diagnosis and management of this disease has been performed. Early identification of the etiology of PLPM is critical and can aid in appropriate management, referrals, and subsequent treatment. This article presents a case of PLPM along with a review of the literature relevant to PLPM including key elements of diagnosis and treatment.