Nickan Research Institute Journal of Renal Endocrinology 2423-6438 8 1 2022 01 01 Von Hippel-Lindau; A 47-year-old woman with renal cell carcinoma and multiorgan involvement e25065 e25065 10.34172/jre.2022.25065 EN Aidin Tarokhian https://orcid.org/0000-0002-6168-5168 Arash Dehghan https://orcid.org/0000-0001-9914-8862 Sohrab Kulivand https://orcid.org/0000-0002-1195-645X Shiva Borzouei https://orcid.org/0000-0001-6826-9872 Journal Article 10.34172/jre.2022.25065 2022 10 09 2022 12 18 Von Hippel-Lindau (VHL) is a rare autosomal dominant genetic disease. Affected individuals manifest clinically with tumors involving multiple organs including hemangioblastoma of the central nervous system and retina, cystic kidney disease, renal cell carcinoma (RCC), pheochromocytoma, and pancreatic neuroendocrine tumors. Its diagnosis is based on genetic testing and clinical criteria in suspected patients. Treatment principles are to save organs and periodic surveillance for in-time tumor detection and appropriate follow-ups. This case report presents a 47-year-old woman with five years history of abdominal pain alongside long-standing headaches and progressive hearing loss. On imaging, multiple suspicious tumors throughout her body were detected and she proceeded to surgical resection. She was finally diagnosed with RCC, central nervous system tumor hemangioblastoma, and pancreatic cyst. The patient underwent a right-sided nephrectomy and Sunitinib therapy. She is continuing therapy and currently does not have any sign of metastasis. Due to her constellation of tumors VHL disease was suspected. Although she did not have any positive family history, the VHL diagnosis was conducted based on clinical criteria. The case emphasized the importance of timely recognition and evaluation of suspected cases and evidence base surveillance. Physician familiarity with the underestimated disease is of paramount importance.