Frontotemporal lobar degeneration (FTLD) is a neurodegenerative disease that affects frontal and temporal regions of the brain. Two proteins indicated in the pathology are tau and the recently discovered TDP-43. Major manifestations include progressive aphasia and a disorder of social comportment. The diagnosis of a patient includes a detailed cognitive exam, clinical testing, and neuroimaging techniques. The current goal of therapy for FTLD is symptomatic management with medications borrowed from other conditions. Nonpharmacologic management such as behavioral interventions and environmental engineering are also efficacious in optimizing quality of life.