Birt-Hogg-Dube syndrome (BHD) is a rare autosomal dominant inherited cancer syndrome characterized by benign tumors of the hair follicle (fibrofolliculoma), pulmonary cysts, spontaneous pneumothorax and increased risk for renal carcinoma. In developing countries like India where affordability and facility for FLCN BHD gene study are not available, presence of skin lesions, pulmonary cyst and renal hybrid tumor comprising of both oncocytoma and chromophobe renal cell carcinoma can contribute for diagnosis.