Background: Increasing numbers of young people with severe neurological impairment are suffering from gastrointestinal symptoms, which may result in nutritional failure and ultimately death. Gastrointestinal dystonia is a recently described clinical diagnosis amongst patients with severe neurological impairment, and no systematic review of existing evidence currently exists. Aim: To conduct a systematic review of existing evidence for the management of gastrointestinal dystonia in children and young people with severe neurological impairment and palliative care needs. Method: A systematic review assessing pharmacological and non-pharmacological treatments was undertaken using standard Cochrane methodology. We searched Cochrane CENTRAL, MEDLINE, EMBASE, and PsycInfo. All databases were searched from inception, and no language restrictions were used. Results: 1580 references were identified. After abstract screening, 56 references were reviewed at full text, and a case report and case series were identified for inclusion. Low-quality, indirect evidence exists for the management of gastrointestinal dystonia, including symptom management, hydration and nutrition decisions, and end-of-life care. Conclusions: There is a paucity of existing evidence directly relating to gastrointestinal dystonia, but low-quality indirect evidence from studies of children with severe neurological impairment and gastrointestinal symptoms exist, which may begin to inform clinical practice.