Hemolymphangioma is a rare disease with malformation of both lymphatic and vascular vessels. Few cases of hemolymphangioma occurring in the rectum, small intestine, pancreas, esophagus, and other organs have been reported. Nevertheless, multiple hemolymphangioma of the visceral organs are extremely rare. We report a 25-year-old female with a significantly enlarged spleen full of multiple-rounded lesions. Curiously, the splenic flexure and even retroperitoneum had many lesions. The patient recovered well after splenectomy and the pathologic diagnosis of spleen was hemolymphangioma with abnormal lymphatic and blood vessels with polycystic spaces.Usually, it is hard to cure this disease. We should take much more consideration into the diagnosis, treatment, and even pathogenesis, even though it is a benign lesion.