In a 34 year-old woman complaining of right upper quadrant pain and having mildly elevated total bilirubin, the imaging investigation revealed a liver lesion with characteristics of focal nodular hyperplasia, measuring 3.8 cm, at the confluence of the hepatic veins. The mass was obstructing the left and middle hepatic veins and nearly obstructing the right hepatic vein. Dilation of the splenic vein with development of retropancreatic varices, splenomegaly and free abdominal fluid were also present. The patient underwent an uncomplicated left hemihepatectomy. Patients postoperative total bilirubin was normalized. Tomographic imaging three months after the liver resection revealed resolution of all the Budd-Chiari radiographic signs. This is a report of a case where a hepatic focal nodular hyperplasia, despite its benign nature, required extensive and urgent surgical intervention due to its location and potential dangers secondary to the development of portal hypertension.