Human cystic echinococcosis is a zoonosis caused by the larval cestode Echinococcus granulosus. Clear cell renal carcinoma is the most common pathological type of renal cell carcinoma. Echinococcosis complicated with carcinoma is rarely reported. Here, we reported a female patient with echinococcal cyst of the liver accompanied with clear cell renal carcinoma. This 27-year-old woman was admitted for abdominal pain. The serological testing of hydatid cyst was positive and levels of tumor markers were within the normal range. The computed tomography and histological findings confirmed hepatic echinococcal cyst complicated with renal carcinoma of kidney. Preoperative liver function was grade A. The patient underwent pericystectomy of liver hydatid cyst and partial nephrectomy. No recurrence was found at 1 year of follow-up. Liver hydatid complicated with renal cell carcinoma is rare, which should be differentiated from liver metastasis of renal cancer. Surgical resection is the optimal treatment. This case may provide insight for the diagnosis and research on the co-occurrence of tumor and hydatid cyst.