Hiccups (singultus) usually present as a common annoyance lasting for short periods. Rarely, they may be the harbinger of a serious disease. We present the case of a 19-year-old man in which intractable hiccups was the first and most prominent symptom of a serious underlying neurologic disorder. The patient had been examined by his pediatrician, and despite multiple medical regiments and physical maneuvers, his symptoms persisted. A thorough head and neck examination revealed a right-sided vocal cord paralysis. This finding prompted obtaining a magnetic resonance imaging scan, which demonstrated a type I Arnold-Chiari malformation associated with a large cervicothoracic syringomyelia. The patient was referred to the neurosurgical service and subsequently underwent a ventriculoperitoneal shunt placement. There was considerable initial improvement in his neurologic status and cessation of the hiccups. However, the symptoms recurred within 1 month. The case report as well as a brief review of the relevant pathophysiologic and etiologic considerations and several treatment modalities for hiccups is presented.