Introduction: Myxoma is a rare benign kidney tumor. Only 18 cases have been described in the literature. We report a new case of renal myxoma that arises from the renal parenchyma. Case presentation: A 56-year-old man, medically free, presented complaining of intermittent vague left-side abdominal pain for 1year, otherwise no history of gross hematuria or lower urinary tract symptoms. Clinical examination revealed a soft, non-tender abdomen. All laboratory investigations were within normal. Abdominal and pelvic computed tomography scan with contrast revealed a large mass located in the upper pole of left kidney with the possibility of renal cell carcinoma. A laparoscopic-assisted left robotic radical nephrectomy was performed. Microscopic examination revealed renal myxoma. Conclusion: Renal myxoma is a very rare benign kidney tumor with excellent prognosis. Considering its rarity, it is important to recognize this entity to avoid diagnostic errors with other neoplasms with prominent myxoid features.