Introduction: Myelodysplastic syndrome (MDS) is a clonal hematopoietic disorder with a risk of progression to acute myeloid leukaemia. This case report discusses a rare instance of spontaneous splenic rupture in a patient with MDS. Case Report: A 76-year-old male with a history of MDS presented with acute abdominal pain and was found to have a splenic rupture. Despite declining chemotherapy, the patient underwent an emergency splenectomy after a CT scan revealed hemoperitoneum. Post-surgery, the patient experienced hypovolemic shock, necessitating a second operation. Discussion: Splenic rupture in MDS is uncommon and its pathogenesis is not fully understood. Management of MDS is complex, often requiring a multidisciplinary approach. This case emphasizes the importance of considering patient preferences in treatment decisions and highlights the role of next-generation sequencing in understanding MDS pathogenesis. Conclusion: The case underscores the need for vigilance in managing MDS due to potential complications like splenic rupture. It also illustrates the importance of personalized care and advances in genetic profiling in predicting prognosis and guiding treatment in MDS.