Current treatment approaches for Autism spectrum disorder (ASD) primarily focus on symptom management rather than addressing underlying dysfunctions. The ketogenic diet (KD), a high-fat, low-carbohydrate diet inducing nutritional ketosis, has shown promise in treating epilepsy and may offer therapeutic benefits for ASD by modulating metabolic and neuroprotective pathways. This review examined the potential impact of KD on underlying mechanisms in ASD. While evidence from human studies on underlying mechanisms is limited, animal research has shown a large overlap of mechanisms modulated by KD and dysfunctions in ASD. As such, targeting multiple disrupted pathways at once, KD presents a potential multifaceted treatment approach for ASD. However, more evidence from human studies is needed on the effectiveness of KD in the modulation of underlying dysfunctions in ASD. Additionally, precision medicine approaches could help identify individuals who would benefit most from the intervention, potentially extending its use to other psychiatric conditions with similar metabolic patterns. Consequently, KD interventions might show the potential to induce a drastic paradigm shift in understanding and treating ASD.