BackgroundPeliosis is a rare condition with anatomopathological characteristics that affect the liver. However, splenic peliosis is even more unique and rare. Patients with such abnormality usually exhibit no symptoms. Moreover, this is a lethal condition due to the high probability of splenic rupture accompanied by shock.Case presentationWe present a case of a 29-year-old Arab female who was admitted to the hospital with severe upper abdominal pain that started 1 week from the date of admission, associated with nausea, anorexia, low-grade fever, and vomiting, with no past medical history or comorbidities. A computerized tomography scan with contrast showed intraperitoneal free fluid along with multiple hypodense splenic cysts. Hence, an emergent exploratory laparotomy with splenectomy was performed. Splenic peliosis was confirmed by the histopathological examination.ConclusionFurther investigations are warranted if peliosis is confirmed in one organ, for example, the liver, to detect its presence in any other potential organs that can be affected by peliosis. Splenic peliosis is extraordinarily rare. Furthermore, such a disease has no established management plan. Definitive treatment is surgical. Many aspects of splenic peliosis remain puzzling requiring more research in the near future.