Erdheim-Chester Disease Presenting as Complete Heart Block: A Case Report

Erdheim Chester disease should be considered in patients presenting with unexplained conduction abnormalities and infiltrative cardiac findings.• Management of cardiac Erdheim Chester disease requires a multidisciplinary approach involving cardiologists, radiologists, oncologists and pathologists, and therapeutic strategies include interferon-alpha and targeted therapies such as BRAF and MEK inhibitors.• Improved risk stratification for conduction disorders and dedicated electrophysiological management are important in patients with cardiac Erdheim Chester disease, as atrioventricular block may occur even during treatment with targeted therapies such as vemurafenib, highlighting the need for close monitoring and follow-up.