Hepatic adenomatosis was first described in 1985 by Flejou et al as multiple adenomas, arbitrarily more than 10, in an otherwise normal liver parenchyma. Several authors have suggested that it is a distinct entity from hepatic adenoma, which is predominantly seen in young women taking oral contraceptives. Although considered a benign disease, it can be associated with potentially fatal complications such as malignant transformation and intraperitoneal hemorrhage due to rupture. Although its etiology and natural history have not been fully elucidated, germline mutation of hepatocyte nuclear factor 1alpha, which is associated with maturity-onset diabetes of the young type 3, has recently been implicated in a subset of cases. Currently, there is no consensus on patient management. However, surgical removal of large lesions may significantly improve symptoms and reduce the risk of complications. Genetic counseling may now play an important role in case management.