End stage renal disease (ESRD) and acquired cystic renal disease (ACRD) are characterized by structural remodelling through chronic inflammation and by frequent development of cancers. So-called eosinophilic-vacuolated and chromophobe-like renal cell carcinoma develop exclusively in ACRD kidney. Previous Affymetrix array analysis detected GPR87 as one of the highly expressed genes in ESRD/ACRD kidneys. Experimental and clinical studies suggested a correlation between activation of GPR87 signalling and proliferation and migration of tumour cells. In this study we have analysed normal and ESRD/ACRD kidneys and related tumours for GPR87 expression by PCR, RT-PCR, and immunohistochemistry. Immunohistochemistry revealed a strong GPR87 expression in proliferating epithelial cells in ESRD/ACRD kidneys and as well as in cells of eosinophilic- vacuolated and chromophobe-like renal cell carcinoma. Our study suggests that GPR87 signalling play an important role in structural remodelling of ESRD/ACRD kidney and development of ACRD-associated tumours with unique histology.