Functional neurological symptom disorder (FNSD) is characterized by neurological symptoms that are unexplained by other traditional neurological or medical conditions. Both physicians and patients have limited understanding of FNSD, which is often explained as a physical manifestation of psychological distress. Recently, diagnostic criteria have shifted from requiring a preceding stressor to relying on positive symptoms. Given this shift, we have provided a review of the etiology of FNSD. Predisposing factors include trauma or psychiatric symptoms, somatic symptoms, illness exposure, symptom monitoring and neurobiological factors. Neurobiological research has indicated that patients with FNSD have a decreased sense of agency and abnormal attentional focus on the affected area, both of which are modulated by beliefs and expectations about illness. Sick role and secondary gain may reinforce and maintain FNSD. The integrated etiological summary model combines research from various fields and other recent etiological models to represent the current understanding of FNSD etiology. It discusses a potential causal mechanism and informs future research and treatment.