Frontotemporal lobal degeneration (FTLD) is a clinically heterogeneous group of sporadic and familial neurodegenerative diseases characterized by dementia, alteration in language and/or behaviour, loss of executive skills and sometimes Parkinsonian features resulting from degeneration predominantly affecting the anterior frontal and temporal regions of the brain. Three main clinical subtypes including frontotemporal dementia (FTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA) have been described depending on the clinical phenomenology, the areas of the brain where the disorder begins and where the most extensive degeneration occurs. We describe a case of frontotemporal dementia in a 58 year old Nigerian woman and also review the current literature. Recent genetic studies have expanded the frontiers of knowledge about FTD while the search for appropriate drug treatments continues.