The hepatic atrophy and hypertrophy complex has been described in a selected group of nine patients with benign bile duct stricture. The clinical features common to this group were a high biliary stricture and a long-standing history of cholangitis and intermittent jaundice. A history of multiple surgical procedures and associated vascular damage or portal hypertension is strongly suggestive of the atrophy and hypertrophy complex. The radiologic criteria for the diagnosis of this condition are presented. Computerized tomography and HIDA scintigraphy were valuable as noninvasive means to diagnose lobar liver atrophy. The atrophy and hypertrophy complex described herein poses significant therapeutic problems and demands approaches other than those normally applicable for high biliary strictures. A combined surgical and radiologic approach with additional interventional radiologic procedures may be appropriate in patients in whom hilar anastomosis is difficult or impossible.