DOI: 10.36347/sjmcr.2016.v04i09.016

Pages: 684-686

Von Hippel Lindau syndrome is a scarcely reported disease, which is multi-system familial tumor syndrome. It follows autosomal dominant type of inheritance pattern. VHL syndrome is usually characterized by presence of hemangioblastoma in various places like CNS and retina. Other manifestations in VHL syndrome are renal cell carcinoma, pancreatic benign cyst and solid tumor, epididymal tumor, cystic lesion of ovary and other viscera, neuro endocrine tumor of pancreas etc. In this article we present a case of VHL syndrome, which came to us with primary complain of menorrhagia and dyspareunia. On further enquiry, patient gave history of multiple episodes of headache and dyspepsia. Radiological investigations showed multiple cysts in pancreas, kidney and ovary. By method of EUS, biopsy of pancreatic cyst was taken which came benign. We report this case of VHL, which presented to us with primary complain of abnormal uterine bleeding, a very rare presentation of this rare disease.