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Mucormycosis is a rare fungal infection that often causes rhinocerebral disease. However, there have been rare cases of mediastinal involvement. These patients remain a therapeutic challenge and mortality in this group is very high. We report a case of mediastinal mucormycosis with invasion of the heart and right lung in a patient with chronic granulomatous disease (CGD) and also review the available literature on mediastinal mucormycosis.