Marta, Patrizia and Albert just published a new review on how mitochondria may act as a trojan horse in neurons. This is because, due to their bacterial origin, mitochondria have a high immunogenic potential. Indeed, mitochondria have been identified as an intracellular source of molecules that can elicit cellular responses to pathogens. The problem is that compromised mitochondria can release mitochondrial content into the cytosol, triggering an unwanted cellular immune response that can cause the degeneration of the cell. In this review, Marta, Patrizia and Albert provide new insights on mitochondria-driven inflammation as a potential therapeutic target for neurodegenerative and primary mitochondrial diseases.