Frontotemporal dementia (FTD) and related conditions are often considered daunting because of the numerous inter-related clinical syndromes and their apparently heterogeneous pathologic substrates. Although the labyrinthine complexity of the disease seemingly continues to grow, recent discoveries have made the maze of FTD somewhat more navigable, spurring new interest in the field. This review begins by surveying the fascinating clinical presentations of these conditions and the few currently available treatments, then turns to recent progress in understanding the pathophysiology of FTD. Among the important advances surveyed are clinicopathologic correlations that enable prediction of the pathologic substrate of certain clinical subtypes, and genetic studies that have been particularly fruitful in identifying new causes of FTD. Copyright © 2006 by Current Science Inc.