Inhibitory GABAergic neurotransmission is strongly altered in humans in several pathological conditions such as Huntington’s disease (HD) and mesial temporal lobe epilepsy (MTLE). Loss of neurotrophic support from cortical neurons triggers the death of striatal GABAergic medium spiny neurons in HD, whereas the balance of excitatory and inhibitory connections is distorted in the hippocampus in MTLE. Autologous cell grafts have emerged as a promising therapeutic option for HD due to their dual action: secreting neuroprotective factors and restoring GABAergic connections within the neural circuitry. Both mechanisms may contribute to alleviating or delaying the onset and progression of neuropathological conditions with loss of inhibitory input. In this chapter, we describe the potential use of human dental pulp stem cells (hDPSCs) and their differentiation toward a neuronal-like GABAergic phenotype for their potential use either on in vitro models or in vivo intracranial grafts. hDPSCs are a source of neural crest stem cells with fewer ethical concerns than other sources of human stem cells, making them ideal for autologous brain cell therapy. The first part of this work focuses on refining existing protocols for differentiating functional neuron-like cells from hDPSCs by introducing retinoic acid (RA) and potassium chloride (KCl) pulses over periods of 21 or 60 days, with the resulting GABAergic cells characterized through immunofluorescence analysis. In a second part, we describe the protocol to graft them and assess their location into the host brain, showcasing the potential of use of this easily accessible human stem cell source for brain tissue engineering and possible HD therapy.