Background: Hemolymphangioma is a rare vascular and lymphatic malformation. To the author’s knowledge, no cases of hemolymphangioma in the kidney have been reported in the literature. Case presentation: We herein report a case of a 68-year-old man with low back pain. NO abnormalities were revealed in laboratory data including liver, renal function and blood and urine sediments. Ultrasonography showed a huge separated cystic mass in the upper part of left kidney. In contrast-enhanced ultrasound(CEUS) , the lesion of the kidney was hyper-enhanced in the hyper-enhanced in the cortical phase, and hypo-enhancement in medulla and delayed phase. Nephrectomy was performed. The pathologicaI diagnosis was a hemolymphangioma of thekidney. His postoperative course was uneventful and he was discharged on the 12th d after surgery. Conclusions: Although rare, hemolymphangioma should be considered a differential diagnosis for masses occurring in the kidney. Complete surgical resection is the treatment of choice and affords a good prognosis.