BackgroundAbsence of portal bifurcation is an extremely rare anomaly that should be recognized preoperatively, especially prior to a major hepatectomy.Case presentationA 45-year-old woman presented with abdominal pain, and abdominal computed tomography (CT) revealed dilatation of both the common bile duct (CBD) and intrahepatic bile duct (IHBD). Endoscopic retrograde cholangiopancreatography (ERCP) showed CBD and IHBD stones (B2 and B4). The CBD stones were removed, but the IHBD stones could not be, yet there was no evidence of malignancy at the site of IHBD stenosis. Enhanced CT revealed a dilated IHBD, while three-dimensional CT images showed the left portal vein running through the ventral side of the middle hepatic vein, which was diagnosed as the absence of portal vein bifurcation (APB). Laparoscopic left hepatectomy was successfully performed using real-time indocyanine green (ICG) fluorescence imaging.ConclusionSurgeons should be aware of the possibility of APB, a rare portal vein anomaly, before performing major hepatectomy. Real-time ICG fluorescence imaging may be helpful to ensure the precise anatomy of the liver during laparoscopic surgery.