Hypovolemic shock and spontaneous splenic rupture (SSR) are unusual fatal complications in the uraemic patients. We described a case of SSR in a 29-year-old hemodialysis (HD) patient. The diagnosis was confirmed with the acute abdomen picture included the left upper abdominal and left shoulder pain together with shock and ultrasound findings. The splenectomy was performed. The postoperative course was uneventful and the patient was discharged from hospital on the 5th day. The mechanism of the rupture is still unknown, but the rupture and subcapsular haematomas of spleen may be induced by the uraemic coagulopathy, the use of anticoagulants during HD, malignant hypertension and unrecognised microtrauma. Splenic rupture should be considered in any patient with abdominal pain and shock, regardless of a history of trauma or previously known risk factors for spontaneous rupture. An aggressive multidisciplinary approach to the management of these patients may decrease the mortality rate.