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Giant right atrial thrombus associated with ICD lead externalized conductors: a case report. Introduction: Narrow calibre ICD leads are prone to present insulation defects and conductor externalization. Close follow-up of these leads is recommended but as long as their electrical function is maintained, no prophyllactic replacement or extraction is advised. Although the risk of thrombus formation involving externalized conductors has been described, this risk seems considered as negligible compared with the risk of a prophylactic lead extraction. However, when an intracavitar thrombus is identified, the safest therapeutic approach remains undetermined. Case presentation: In the present clinical vignette, we describe the case of a giant thrombus developed along the externalized portion of an electrically functional ICD lead. In this case, the thrombus was successfully treated with a systemic oral anticoagulation. Discussion: This case report supports the concept of a prolonged anticoagulation for both the diagnosis and the long-term treatment of thrombus developed along externalized ICD leads, in particular when the patient prefers to avoid or postpone the risk of a trans-venous lead extraction.