Umbilical hernia with dilated paraumbilical collateral vein as its content is rare. Diagnosing this condition is extremely crucial as rupture of the herniated umbilical vein during herniorrhaphy in a patient with ascites can be a fatal complication. Here we are reporting a case of a 37-year-old male who presented with complaints of abdominal distension, hematemesis, umbilical hernia with pulsations felt over the herniated sac. On ultrasonography, features of liver cirrhosis, portal hypertension and umbilical hernia with a herniated dilated vessel showing venous waveform on Doppler were seen. Contrast enhanced computed tomography (CECT) showed signs of liver parenchymal disease and dilated left paraumbilical vein coursing just beneath the anterior abdominal wall towards umbilicus with herniation through the umbilical hernia. Porto-systemic collaterals from this were then seen to be draining into right common femoral vein. Looking at the clinical and radiological features – diagnosis of Cruveilhier Baumgarten syndrome was observed