Chronic myelomonocytic leukemia (CMML) is a myelodysplastic syndrome (MDS) characterized by prominent monocytosis and an increase in bone marrow monocyte precursors in addition to dyshaematopoietic features (1). Extrahaematological manifestations including cutaneous, neurologic, and rheumatic symptoms have been recorded in association with CMML. Here, we report the first observation of renal, adrenal and perirenal involvement in CMML which presented as a kidney tumor.