Epilepsy in frontotemporal dementia is considered to be less frequent than in Alzheimer’s disease. We report two cases of patients with non-convulsive status epilepticus associated with behavioral variant frontotemporal dementia. In the first case, status epilepticus was the first symptom of the disease, and consisted of loss of consciousness and mutism. In the second case, status epilepticus led to a clinical worsening one year after the diagnosis. Our study highlights the importance of suspecting non-convulsive status epilepticus in patients with frontotemporal dementia, and including frontotemporal dementia within the differential diagnosis of new-onset seizures.