Familial temporal lobe epilepsy is a heterogeneous genetic syndrome, initially recognized in studies with twins. In this condition, many members of a family are affected by psychic or autonomic auras and focal seizures with altered consciousness. We describe two brothers, children of non-blood parents, with different phenotypes of familial temporal lobe epilepsy and drug resistance who were referred to our center for epilepsy and functional neurosurgery. The family, of Mayan descent, has a history of epilepsy in other relatives. Epilepsy surgery was performed on the two brothers while remaining seizure-free today. The histological findings in both cases were consistent with cortical focal dysplasia IIIa. It is the first Latin American report of siblings with evidence of a correlation between familial temporal lobe epilepsy and histological changes suggestive of cortical focal dysplasia IIIa.