Paragangliomas (PGs) are rare, benign neuroendocrine tumors that originate from neuroectodermal tissues. PGs arising from the ear canal are extremely rare, with only a few cases reported to date. The common symptoms of ear canal PGs include ear discharge, ear fullness, and hearing loss. This article describes unusual manifestations of ear canal PGs otitis media with Bezold and Luc’s abscesses, in an immunocompromised young adult. The pathogenesis of the disease is described. In addition, determining the origin of PG in this case was not straightforward compared to previously reported ear canal PGs. The derivation of the diagnosis is further discussed.