Ethylene glycol (EG) and diethylene glycol (DEG) intoxication, defined as > 20 mg/dL in plasma, can cause severe morbidity and mortality due to their metabolism into toxic metabolites via alcohol dehydrogenase (ADH). In August 2022, Indonesia experienced a surge in acute kidney injury (AKI) cases in children, initially misdiagnosed as multisystem inflammatory syndrome in children (MIS-C). Following a World Health Organization (WHO) alert on EG/DEG-related AKI in Gambia, toxicology screening confirmed EG/DEG contamination in syrup-based medications. This study evaluates the efficacy of fomepizole in treating 19 pediatric patients with EG/DEG intoxication. All patients received fomepizole based on American Academy of Clinical Toxicology (AACT) guidelines, with additional hemodialysis in severe cases. Laboratory assessments revealed metabolic acidosis, elevated urea and creatinine levels, and hepatic dysfunction. Fomepizole administration led to significant renal function improvement, with 14 patients recovering and five fatalities (mortality rate: 26.3%). While fomepizole showed transient hepatotoxic effects, overall hepatic function improved. Early recognition of EG/DEG intoxication is critical, as clinical symptoms may not follow a predictable progression. Prompt administration of fomepizole significantly reduces morbidity and mortality, making it an essential intervention for EG/DEG poisoning. © The Author(s) 2025.