Trigeminal neuralgia (TN) is paroxysmal, lancinant pain often described as an "electric wave" by patients, with involvement of the divisions of the fifth cranial nerve. Demyelinating, compressive, ischaemic diseases are involved in the physiopathology of TN, but there are some cases without explanation. Familial TN (FTN) is a rare condition, about 1%-2% of all TN cases, while sporadic cases are the most common. To date, there have been about 126 reports of FTN. We describe the case of a 66-year-old man who had been complaining for 3 years of right-side paroxysmal lancinating pain in the second division of the fifth cranial nerve. A brain MRI with angiographic sequences did not show neurovascular conflicts or other pathological conditions. The patient had a family history of TN, which had been diagnosed in 3 other family members (father, sister and first cousin), who had undergone medical or surgical treatment for TN. There was no family history of hypertension, metabolic disorders, neurological or traumatic diseases. Animal studies have shown a probable involvement of genes codifying for calcium channels as the starting alterations in trigeminal excitability. Our FTN could be a good model to investigate the role of gene mutations in this condition.