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Introduction: Wegener's granulomatosis (WG) involves mainly upper and lower respiratory tract and the kidneys. Cardiac involvement is not common. Case Report: This is a report of a 34-year-old, non-smoker male with very unusual presentation of (WG) namely a combination of large pericardial effusion, cardiac tamponade (without uremia) and conduction defects, all of which responded very well to immunosuppressive therapy without the need for surgical intervention. Conclusion: Cardiac complication of WG may start early and early diagnosis and management improves the outcome and decrease, overall mortality.

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