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Background: Sinusoidal obstruction syndrome (SOS)/veno-occlusive disease (VOD) is generally associated with hematopoietic cell transplant (HCT), but little is known about this condition outside the HCT setting. This systematic review examines the burden of illness and current management approaches in non-HCT SOS/VOD. Methods: We searched Embase, Medline, and grey literature sources for non-HCT SOS/VOD studies published 2002–2023. Inclusion criteria were studies of any design reporting incidence, diagnosis, underlying disease and any ongoing treatment at the time of SOS/VOD onset, management of non-HCT SOS/VOD, clinical burden, health-related quality of life, healthcare resource use, costs, and patients’ unmet needs. Studies investigating pulmonary VOD or SOS/VOD related to the ingestion of pyrrolizidine alkaloids were excluded.Two authors independently screened results, extracted data, and assessed the methodological quality of studies using the Motheral scale for retrospective studies, Newcastle-Ottawa scale for prospective studies and case control studies, the Cochrane risk of bias tool for randomized controlled trials, and the Joanna Briggs Institute critical appraisal tool for case series. Results were synthesized narratively. Results: Ninety-two studies were included; 57 % were retrospective cohort studies and 70 % were conducted in the US or Europe. The study populations included hematological and solid tumor cancers, various indications for liver transplant, Wilms’ tumor, and transfusion-dependent beta thalassemia. Non-HCT SOS/VOD occurs most frequently in people with colorectal liver metastases (CRLM), acute lymphoblastic leukemia (ALL), and acute myeloid leukemia (AML). Approximately 35 % of oxaliplatin-treated CRLM patients and 5 % of ALL and AML patients have non-HCT SOS/VOD. Diagnosis varies according to initial disease setting. Defibrotide is the most frequently reported treatment. Most studies did not clearly report their data sources or methods of outcome assessment. Conclusion: Non-HCT SOS/VOD occurs in diverse disease conditions, therefore guidelines on diagnosis and treatment are needed to optimize management in clinical practice.