Background: Wegener's Granulomatosis (WG) is classically characterized by the triad of sinonasal, pulmonary, and renal manifestations. However, a growing body of research has demonstrated that atypical head and neck pathologies including retropharyngeal and parapharyngeal lesions are often the presenting feature of patients found to have WG.
Case: This report describes the hospital course of a patient who presented with a parapharyngeal mass with secondary superior cervical chain compression and Lemierre's Syndrome. We discuss how a stepwise approach involving a multidisciplinary team led to the diagnosis of WG.
Conclusions: To our knowledge, this report is the first description of a patient presenting with a parapharyngeal mass causing superior cervical chain compression with simultaneous Lemierre's Syndrome who was ultimately diagnosed with WG. We highlight how the early consideration of WG in patients with atypical head and neck lesions refractory to multiple treatment regimens can led to an expeditious diagnosis and the coordination of appropriate short-term and long-term care.