Systemic lupus erythematosus (SLE) is a multisystemic, autoimmune, inflammatory disease. Gastrointestinal (GI) involvement, extensively described in adults, is less characterised in paediatric-onset SLE (pSLE). The aim of the present narrative review was to provide a comprehensive summary and update on GI involvement in pSLE. A literature search on PubMed and EMBASE was conducted to identify original articles, reviews, case series and editorials published in English from 2000 to 31 August 2020. Based on this, we reported the prevalence, pathogenetic mechanisms, clinical issues, diagnostic tools and management of each form of GI involvement in pSLE. Lupus enteritis is the most frequent type of GI involvement in pSLE, followed by intestinal pseudo-obstruction, protein-losing enteropathy, hepatic disease and acute pancreatitis. The most common presenting GI symptoms are non-specific and include abdominal pain, anorexia, nausea, vomiting. In most cases, they are associated with other clinical and laboratory manifestations of SLE. The complications of GI involvement, including perforation and intestinal infarction, can be life-threatening. Laboratory findings and imaging studies can help to rule out non-SLE related causes for GI manifestations and to reveal typical features of the single forms of GI involvement. Early diagnosis and treatment are crucial to improve prognosis and avoid unnecessary surgery. Most SLE GI manifestations respond well to glucocorticoids and immunosuppressants. In conclusion, GI involvement is frequent in pSLE and its diagnosis and management can be a challenge for clinicians. In view of the limited available data, further studies are needed to better explore the prevalence, prognosis and treatment recommendations for GI involvement in pSLE.