Pediatric literature and guidelines of treatment options for right atrial thrombosis (RAT) are lacking; thus, this review summarizes the available literature on RAT in infants and children. Medline search identified 35 publications, with 27 prospective or retrospective case series included for data analysis. A total of 122 cases of RAT were identified. The mean age of patients is 3.58 years (n = 86) with a strong predominance in the neonatal and infancy period. Ninety-one percent of cases were found to be associated with central venous catheters, 40.8% are premature neonates, 27.2% are postcardiac surgery patients, and 19.2% have underlying malignancies. Gut failure with total parenteral nutrition given via the central venous catheters occurred in 45.6% of patients. The most frequent presenting symptoms are respiratory distress and arrhythmia, and 56.8% (42 of 74) were asymptomatic. Our study defined high-risk features on echocardiogram as large size, more than 2 cm in any dimension, pedunculated, mobile, or snake-shaped, and mobile. Our result confirmed there is significant difference in the mortality for the high-risk group (16.7%; three of 18) versus the low risk group (0%; n = 32; P = 0.0416). Moreover, none of the asymptomatic patients showed progression in disease or died. Asymptomatic and hemodynamically stable patients with RAT who are at low risk are associated with good prognosis irrespective of treatment. We recommended removal of central venous line if possible, with or without anticoagulation for this group of patients. Systemic anticoagulation therapy should be given to all high-risk or symptomatic RAT patients. Surgical thrombectomy or thrombolytic therapy carries significant risk and should be considered individually.