Frontotemporal dementia (FTD) is increasingly recognized as an important type of degenerative dementia but satisfactory pharmacological treatment has not yet been established. We examined the clinical effects of aripiprazole, a new antipsychotic with partial agonistic properties at serotonin 5-HT(1A) and dopamine D(2) receptors, in parallel with cortical glucose metabolism changes. We conducted a follow-up investigation of clinical status and (18)F-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET) in a 73-year-old male patient with FTD over a 13-month period. Under conventional drug treatment during the first 12 months a marked increase in dementia symptoms was observed. Frontal lobe glucose metabolism clearly decreased during this time period. Under consecutive treatment with aripiprazole a significant and stable improvement of clinical symptoms could be registered, while disturbed frontal glucose metabolism increased significantly. According to this case experience, further investigations should be undertaken to ascertain whether aripiprazole or other atypical antipsychotics with properties to improve impaired dopaminergic transmission in frontal brain regions could qualify for therapy of FTD.