We presented a four-year-old girl with Tay-Sachs 
disease who had thrombus in the right atrium. The patient 
was admitted to hospital with cardiopulmonary arrest and 
aspiration 
pneumonia was diagnosed. Intracardiac 
thrombus was detected during rutine echocardiographic 
examination. There was no underlying genetic 
prothrombotic predisposition in this patient. The 
guidelines of treatment for intracardiac thrombus in 
children are lacking in the literature andthe experience in 
managing these patients is limited. We aimed to to discuss 
the treatment approaches to intracardiac thrombi and to 
emphasize that, development of intracardiac thrombus is 
common in critically ill children and can be treated 
successfully by non-invasive anticoagulation with heparin.