Arterial thrombosis is increasingly recognized in children and is mostly related to the presence of an arterial catheter or an ischemic stroke. Treatment of children with arterial thrombosis varies widely and consists on the administration of the common available anticoagulant und antiplatelet drugs. No evidence-based guidelines are available so far to prefer one treatment approach to another. Data in adults indicate that understanding the pathomechanism and composition of arterial thrombosis is crucial for choosing the most efficient and safe antithrombotic drug. This review will briefly summarize new insights on the pathomechanism and composition of arterial thrombosis in adults and describe available antithrombotic treatment modalities currently used n children.