Central venous catheters are widely used in children with critical illness and chronic disease. These devices are often essential in the delivery of medications and intravenous fluids and in hemodynamic monitoring. Central venous catheter occlusion and thrombosis are common problems in patients using these devices. This article reviews the background, pathophysiology, and incidence of catheter occlusion and catheter-related thrombosis. Diagnostic, preventive, and treatment strategies, along with future research directions, are addressed.