Thyroid disorders are among the most frequent endocrine pathologies with an overall prevalence estimated at 10%. Autoimmunity plays a crucial role in most thyroid dysfunctions, which explains the frequent associations with other autoimmune diseases. Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a necrotizing systemic vasculitis of small-sized vessels specifically associated with antineutrophil cytoplasmic antibodies (ANCA). Thyroid involvement is exceptional and uncommon in association with systemic vasculitis. It has not been previously reported during EGPA. We discuss the possible theoretical mechanisms of thyroid dysfunction that can be seen during this systemic angiitis.