Here, we present a rare case of a patient who developed multiple cranial nerve (CN) palsies over 10 days and was initially suspected of having GPA due to positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA), cranial polyneuropathies, and possible nasopharyngeal involvement but was ultimately diagnosed with IHP due to exclusion of all other known etiologies and treated with high-dose corticosteroids and rituximab leading to resolution of near-majority of neurologic symptoms. The gene discussed is PRTN3; the disease is granulomatosis with polyangiitis.