Thrombosis is an increasingly recognized complication occurring primarily in children with serious underlying conditions and most often associated with intravenous catheters. There are few clinical trials available to guide the decision-making in the diagnosis and treatment of thrombosis. Although there are published guidelines which suggest how to manage such patients, they are based on data from adult studies and uncontrolled pediatric studies and it is unclear how widely these guidelines are utilized in clinical practice. What is clear is that many additional studies are needed to provide the data required to make evidence-based decisions. Nevertheless, patients today are being diagnosed with thrombosis and must be treated. Diagnosis is based largely on various diagnostic-imaging methods. While Doppler ultrasonography is non-invasive and relatively inexpensive, its sensitivity for the upper venous system is poor. Venography/angiography, the most sensitive method to diagnose venous/arterial thrombosis is underutilized due to the need for peripheral venous access for venography and arterial catheterization for angiography as well as interventional radiology. Newer methods such as computed tomography and magnetic resonance imaging are useful for large vessel disease but are not validated in pediatrics. Treatment is based on the methods that are best at restoring circulation rapidly balanced by the risk for bleeding. Both thrombolysis and anticoagulation can be utilized with the circumstances of each individual patient dictating the choice. The field of pediatric thrombosis continues to advance with numerous new studies currently underway or being planned. In the near future, the results of these studies will allow for better management of pediatric patients with thrombosis.