Trigeminal trophic syndrome (TTS) is a rare cause of facial ulceration, which is believed to develop after insult to the trigeminal ganglia or other parts of the peripheral/central nervous system in the trigeminal pathway. The pathogenesis of TTS is poorly understood. Developing a better understanding of TTS will allow early recognition and improved treatment. Although the ulcers develop predominantly on the ala nasi, the literature on ulcer locations is limited. In this article, we review the epidemiologic aspects of TTS, expand on our knowledge of the anatomic location of the ulcers, and discuss current theories for its aetiology and briefly review the approaches to its management.