Hydatid disease is a condition affecting mainly the liver or, to a lesser extent, the lungs. We present an uncommon case of a primary renal echinococcosis in a young man complaining of intermittent hematuria, dull flank pain, and palpable mass in the left lumbar and lateral abdominal area which increases its size over time. After initial self-treatment with painkillers and antibiotics, the patient was referred to a urological clinic, where the physical examination revealed a large tumour mass in the left kidney. Ultrasound showed a large multilocular renal cyst, with a CT highly suspicious for renal echinococcosis (15×12.8×24.2 cm). Serological tests confirmed presence of IgG against Echinococcus. The patient was operated using the lumbar approach and nephrectomy was performed. The man recovered completely after surgery without the need for further treatment.