- Background Desmoplastic trichoepithelioma (DTE) is an uncommon adnexal tumor usually found on the face of young women. Objective We sought to describe the clinical and histologic characteristics of DTE and discuss management strategies for these tumors. Methods We conducted a retrospective case series and review of the literature. Results A series of 17 cases of DTE is reported. Of our patients, 82% were female and half of all these cases occurred on the cheek. Unlike previous reports, the patients in our study were slightly older, with 53% older than 51 years and 71% between the ages of 31 and 60 years at the time of surgery (mean age 49 years, median age 52 years, range 14-81 years). Approximately 38% of the tumors had been present for 1 to 2 years before presentation. The average size of the tumors at the time of surgery was 0.8 × 0.6 cm. All cases were treated with Mohs micrographic surgery. To date, none of the tumors have recurred. Limitations This is a small case series. The referral patterns of our predominantly Mohs micrographic surgery clinic might further bias this study. Conclusion DTE is a benign tumor that has a predilection for the face. With its rapid growth, aggressive histologic features that may be confused with basal cell carcinoma, and tendency to arise in cosmetically and functionally sensitive areas, these tumors should be removed in a manner that assures clear surgical margins, such as Mohs micrographic surgery.