Background. A 69 year-old woman presented to an interdisciplinary medical group with pathological gambling, and went on to develop disinhibition, loss of empathy, and perseverative, stereotyped and ritualistic behavior. An initial neuropsychological evaluation showed selective impairment on the Iowa Gambling Task similar to that of patients with behavioral variant frontotemporal dementia, despite normal performance on standard neuropsychological tasks. MRI scans showed frontal lobe atrophy, which was consistent with findings on hexamethylpropyleneamine oxime single photon emission CT (HMPAO-SPECT). Investigations. Physical examination, neuropsychiatric and neuropsychological assessments, MRI brain scan, HMPAO-SPECT.Diagnosis. Behavioral variant frontotemporal dementia.Management. Pharmacological treatment with the selective serotonin reuptake inhibitor paroxetine for impulsive behavior and carbamazepine to stabilize mood. The patient and her family also received counseling to advise on behavioral and legal issues.