Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) is a form of multisystemic necrotizing vasculitis that has various manifestations. Cardiac involvement is one of the indications of poor prognosis, occurring with different reported prevalence. Due to the heterogeneity of EGPA presentation, its diagnosis relies on clinical condition supported by different investigations and classification criteria. However, large hemorrhagic pericardial effusion is a rare occurrence in EGPA, raising a diagnostic challenge with a wide differential diagnosis. EGPA can also affect other layers of the heart, making its development to perimyocarditis a warning for the progressive nature of the disease. We report a case of EGPA in a young female patient who presented initially with large hemorrhagic pericardial effusion, and later developed perimyocarditis.