Acquired aplastic anemia (AA) in children is a rare, life-threatening disorder characterized by pancytopenia and hypocellular bone marrow. Early referral to specialized centers ensures accurate diagnosis and timely treatment. Differential diagnosis must exclude inherited bone marrow failure syndromes and immune-related disorders using comprehensive genetic and immunologic testing. Hematopoietic stem cell transplantation (HSCT) from a matched sibling donor remains the first-line treatment when available, offering high survival rates. In the absence of a suitable donor, immunosuppressive therapy (IST) with horse antithymocyte globulin and cyclosporine is the standard. Upfront matched unrelated donor transplantation is increasingly used due to improved outcomes. Supportive care, including transfusions, infection prophylaxis, and iron chelation, remains critical. Emerging options such as haploidentical transplantation and thrombopoietin receptor agonists provide alternatives for refractory cases. Despite therapeutic advances, long-term follow-up is essential to monitor clonal evolution and late effects. Personalized, risk-adapted strategies are pivotal to optimizing pediatric AA management.