Venous thromboembolism (VTE) is a leading cause of morbidity and mortality among hospitalized patients, including children. In recent years, it has become clear that hospitalization and critical illness bestow an increased VTE risk in pediatrics and relate to mortality and life-limiting comorbidities. For critically ill children, reported rates of VTE vary by study sampling techniques, presence of inherited or acquired thrombophilia, acute and chronic immobility, underlying illness prompting hospitalization, and clinical factors related to illness severity such as central venous catheterization, length of stay, mechanical ventilation, and patient age. Accordingly, critically ill children with new signs of venous congestion, acute inflammation, or unexplained acute organ dysfunction should be routinely evaluated for VTE. This narrative review summarizes recent and historical literature regarding risk factors, prevention, presentation, treatment, and outcomes of VTE in critically ill children. In addition, we identify knowledge gaps and priorities for future collaborative research on this vital condition. Special attention is given to the clinical trial opportunities, challenges, and ongoing efforts in thromboprophylaxis in critically ill children, including those hospitalized for disease related to novel coronavirus (COVID-19) and multisystem inflammatory disease in children.