Nontraumatic splenic rupture and hematoma are rare in sickle cell disease. We present a case of a 22-year-old Saudi male with sickle cell disease. He presented to our hospital with a history of nontraumatic abdominal pain, hemodynamic instability, and abdominal tenderness, with a large mass extending to the umbilicus. A computed tomography (CT) examination showed splenomegaly and a spleen infarction. The patient was admitted to the intensive care unit (ICU) and stabilized. He was transferred to the regular ward and discharged against medical advice (DAMA). Later on, he presented again with persistent abdominal pain. He underwent splenectomy with cholecystectomy. The patient did well postoperatively and was discharged in good condition. While conservative management is common, operative management should be considered in patient with persistent pain. Splenic rupture has a high mortality rate.