BACKGROUND There is limited information on ipsilateral synchronous papillary renal cell carcinoma (PRCC) and clear cell renal cell carcinoma (CCRCC). Therefore, these rare tumors are often misdiagnosed preoperatively as a single tumor with intrarenal metastasis or some other diseases. Effective management and long-term overall survival might be affected because the prognosis of the two tumors differs. CASE SUMMARY We describe a case of ipsilateral synchronous PRCC and CCRCC with two histological variants in a 72-year-old man, whose mass was found incidentally, with no other chief complaints and vital signs were normal. Initial ultrasound revealed a hypoechoic lobular mass with a volume of 7.8 cm × 4.8 cm × 2.8 cm in the middle to lower pole of the left kidney. A subsequent contrast-enhanced computed tomography scan showed a single endophytic mass of 7.5 cm in diameter. The patient underwent laparoscopic left radical nephrectomy. A final diagnosis of ipsilateral synchronous PRCC and CCRCC was confirmed by pathological examination. There was no recurrence or metastasis after 25 mo follow-up. CONCLUSION We report a case of ipsilateral synchronous PRCC and CCRCC, and review related literature to estimate the prevalence of similar cases. The above descriptions may be expected to help understand the disease, and improve diagnosis in the future.