Autism spectrum disorder is a lifelong neurodevelopmental condition encompassing complex physical and neurological symptoms, including complex sensory symptoms. This review explores the interface between autism spectrum disorder and paediatric ENT. Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses ('PRISMA') guideline, a robust literature search and review was conducted by two researchers. Thirty-four papers were filtered into the final review. Published literature clearly demonstrates potential for autism spectrum disorder to present in the form of auditory and other sensory symptoms to ENT surgeons and audiologists who may not fully appreciate this complex condition. Despite this well-documented link, auditory symptoms, auditory processing disorders and hearing loss within autism spectrum disorder remain poorly understood. Improved recognition and understanding of autism spectrum disorder by otolaryngologists could enable more effective diagnostic and management strategies for autistic children who present with auditory and other sensory symptoms. In light of the current 'autism epidemic,' there is an urgent need for further research on this theme.