Trigeminal neuralgia is the most common craniofacial pain syndrome of neuropathic origin. Although the diagnosis remains based exclusively on history and symptomatology, modern diagnostic techniques, particularly high-resolution magnetic resonance imaging, provides valuable new insight into the pathophysiology of these cases with additional implications for therapeutic strategies. Other neuropathic syndromes affect the trigeminal nerve and warrant different treatments with varied rates of success. Rarely, neuralgias of other cranial nerves mimic trigeminal neuralgia. Finally, it is imperative to distinguish atypical facial pains from these neuropathic syndromes to avoid unsuccessful therapies.