A huge lymphovenous malformation in the retroperitoneum

Vascular malformations are localized or diffuse errors of embryonic development resulting in collections of abnormal vessels with normal endothelium. Many authors have pointed out the confusing terminology of vascular anomalies that has led to improper diagnosis and therapy. This still remains confused in clinical practice, especially in cases occurring at rare regions or presenting atypical radiologic findings. We present a huge retroperitoneal lymphovenous malformation, which exhibited unusual radiologic findings and was difficult to confirm the diagnosis that occurred in a 12-year-old male.