Renal cell carcinoma is the most common type of renal malignancy and it originates from the renal tubular epithelium. Due to the diversity in the histopathological and molecular characteristics, it is typically subclassified into five different categories. Papillary renal cell carcinoma is one subclassification and it includes two variants: sporadic and hereditary. Although the hereditary form comprises a smaller number of cases of papillary renal cell carcinoma, an understanding of the molecular pathways and genetic changes continues to play a significant role in the development of new targeted therapies. Along with recommending appropriate lifestyle modification, further investigation into the molecular pathogenesis of hereditary papillary renal cell carcinoma will continue to be invaluable for the clinical management of renal cell carcinoma. In this article, we discuss a case of the hereditary papillary renal cell carcinoma along with an overview of the disease.