This Case Study describes in detail a case of progranulin-associated frontotemporal lobar degeneration (FTLD). The patient was initially asymptomatic, but developed progressive brain atrophy and deficits on neuropsychological testing before the clinical onset of cognitive impairment. The authors use this case as a springboard to discuss advances in the genetics of FTLD with an emphasis on the importance of serial cognitive testing and neuroimaging.