Malignant kidney neoplasms are quite common in Russia and the world. Clear cell renal cell carcinoma is most common among the malignant kidney neoplasms. There are also other kidney tumors that include tubulocystic renal cell carcinoma (TCRCC). The latter was first identified as an independent tumor entity in the WHO classification only in 2016. The incidence of TCRCC is less than 1%. It is a spongy nodular mass. The cavities are lined with a single layer of cells displaying eosinophilic cytoplasm and a round nucleus, in which a nucleolus is frequently found. The paper describes a case of a patient with TCRCC and clear cell renal cell carcinoma in one kidney. The diagnosis was made using contrast-enhanced ultrasound and magnetic resonance imaging. The patient underwent organ-sparing laparoscopic resection of two renal masses. Both masses had a characteristic structure, as shown in a morphological study. After 3 months, control ultrasound examination revealed no signs of recurrence.