Background: Epilepsy with a genetic background is increasingly being identified. In certain dog breeds, epilepsy occurs with a higher prevalence than the estimate of 1-2% reported in the general dog population.Hypothesis: The Petit Basset Griffon Vendeen (PBGV) experiences an increased occurrence of epilepsy compared to the general dog population.Animals: The target population consisted of all 876 PBGV dogs registered in the Danish Kennel Club from January 1, 1999 to December 31, 2008. The study population included 820 dogs that met the inclusion criteria.Methods: A population study was conducted to estimate the prevalence of epilepsy in the Danish PBGV population. A mailed questionnaire was used to detect possible signs of epilepsy. The information was subsequently validated by telephone interviews of positive and possible positive responders and a negative responder control group, using an extensive questionnaire developed to detect epilepsy. Dogs evaluated as epilepsy positive after the telephone interview were offered a clinical investigation.Results: The prevalence of epilepsy was estimated to be 8.9% (42/471) in the PBGV population. Average age of onset was 26.3 months. Sex and mode of response did not affect the prevalence, but a strong litter effect was seen. Among euthanized dogs, epilepsy was the predominant cause (6/45 = 13.3%).Conclusion and Clinical Importance: Petit Basset Griffon Vendeen dogs experience an increased risk of epilepsy characterized by a relatively early onset and dominated by focal seizures with and without secondary generalization. With an estimated prevalence of 8.9% and substantial clustering within litters, a genetic factor associated with epilepsy is suspected.