KV7 channels have raised an increasing interest in the last years for their important role in brain and heart physiology, and since four of its five so far identified members are related to different hereditary diseases. These channels are expressed in brain, heart, muscle, inner ear, and intestines. They give rise to functionally important potassium currents, reduction of which results in pathologies such as long QT syndrome, neonatal epilepsy or progressive deafness. The talk will be focused on mechanisms of neuronal KV7 channel mutations causing neonatal seizures or peripheral nerve hyperexcitability. Moreover, KV7 channels present attractive pharmacological targets for treatment of diseases characterized by membrane hyperexcitability and the first compound enhancing the activity of neuronal KV7 channels has just been launched for the add-on treatment of focal epilepsy.