Introduction/Background Muir-Torre Syndrome (MTS) is a rare autosomal dominant inherited disease that appears to be a variation of non polyposis colorectal carcinoma-HNPCC, known as Lynch syndrome. Current criteria for its diagnose include at least one sebaceous malignancy and a visceral cancer, most commonly colonic and genitourinary.

Methodology We report a very interesting case of a 73-year old European woman that presented with four different types of neoplasms. These neoplasms encompass two types of sebaceous tumors, as well as vulvar and urothelial cancer.

Results As far as we know, concerning vulvar cancer while coexisting with MTS, this case is the first to be described in literature.

Conclusion Although MTS is very rare, we should pay considerable attention when dealing with more than one malignancy, including sebaceous tumor. Then, we should take a detailed family history as well as advise the family members to undergo genetic counseling about the risk of possible occurrence.

Disclosure Nothing to disclose