We present the case of a patient admitted to our emergency ward with a clinical setting of acute abdominal pain and a history of cavernous lymphangioma, diagnosed in another center by exploratory lapartomy. The patient presented complete analysis including serology tests, as well as an abdominal CT scan that revealed multiple large size retroperitoneal cysts. In view of the clinical symptomatology and results of the tests, a second CT scan was carried out upon admission. As a result of the findings obtained, a second exploratory laparotomy was carried out in which intestinal resection of the perforated jejunal loop and largest cysts was performed. Pathological anatomy diagnosed an intestinal lymphoma associated with enteropathy and abdominal cysts compatible with cavernous lymphangioma. In this work we describe both pathologies, the most characteristic aspects are analyzed and the etiology and possible relation between both entities is discussed.