Two cases of endoplasmic storage disease of liver are described. The liver tissue in each case showed numerous intracytoplasmic hyaline inclusions of varying sizes with formation of ground-glass hepatocytes. These inclusions were pale eosinophilic in hematoxylin & eosin stained sections, and were periodic acid-Schiff and HBsAg negative. Immunoperoxidase studies revealed strong positivity for fibrinogen and complement components C3 and C4 in case 1 and exclusive positivity for fibrinogen in case 2. On electron microscopy, the inclusions appeared as granular or fibrillar material within dilated cisternae of rough endoplasmic reticulum.