Cowden syndrome presenting with trichilemmomas

Cowden syndrome (CS) is a genetic cancer predisposition syndrome that is associated with germline mutations in the phosphate and tensin homologue deleted on chromosome ten (PTEN) tumor suppressor gene. It is characterized by the formation of benign and malignant tumors. Characteristic benign tumors include trichilemmommas, acral keratoses, mucocutaneous neuromas, and oral papillomas. The most common malignant condition include breast, thyroid, and endometrial cancers. We present a case of a 30-year-old woman with CS, who initially presented with trichilemmomas that were misdiagnosed as comedonal acne. Recognition of the presenting features of CS is important to ensure proper referral, management, and treatment for these patients.