The authors report the case of a 45-year-old woman with medically intractable trigeminal neuralgia (TN) in whom a good clinical response to partial sectioning of the trigeminal nerve was attained. No evidence of vascular compression was found intraoperatively. Several other members of her family, involving three generations, also suffered from TN. The treatment of all affected patients is discussed in the context of a literature review in which the controversies surrounding the origins of the disease and treatment options for patients with the familial variant of TN are addressed.