Frontotemporal lobar degeneration (FTLD) is a devastating disease that profoundly changes emotion, self, and personality while initially sparing many aspects of cognitive functioning. This article reviews research that applies methods from basic affective science to obtain a more precise view of FTLD’s impact on emotional functioning. This research indicates that simple forms of emotional reactivity are relatively preserved in the early stages of the disease. In contrast, more complex emotional processes, such as those involved with self-conscious emotions (e.g., embarrassment), emotion regulation, and recognizing emotions in others, are severely impaired. FTLD provides rich opportunities for increasing our understanding of the nature of emotion and of the emotional and social brain. KEYWORDS—emotion; self; dementia; frontotemporal dementia; neurodegeneration A warm, nurturing mother becomes increasingly indifferent to her family; her son comes to her with a serious injury and she shows no concern. A cultured professional begins to make embarrassing social gaffes and seems to neither notice nor care. A conservative businessman becomes increasingly vulnerable to ‘‘get rich quick’’ schemes and seems unaware of how his actions are contributing to his company’s growing debt. These scenarios are typical of those encountered in patients with frontotemporal lobar degeneration (FTLD). Devastating for the lives of patients and their families, FTLD is nonetheless one of the most informative neurological disorders for those interested in human emotion, personality, and the self. FTLD initially spares cognitive functions such as memory and spatial abilities, which are early targets of Alzheimer’s disease (AD). Instead, FTLD hones in on those parts of the brain that determine how we feel, our sense of self, our personality, and how we interact with others. FTLD and AD are both neurodegenerative diseases, but they travel different routes. AD has sometimes been described as a slow journey into darkness, with family members continuing to catch glimpses of the person they knew and loved along the way. In contrast, FTLD progresses much more rapidly, quickly erasing the person once known. The ‘‘new’’ person who emerges in FTLD can still remember, calculate, navigate, and conduct many of the activities of normal life, making the disease seem even more cruel.