Cowden syndrome, a subset of PTEN hamartoma tumor syndrome, is a rare genetic disorder that is inherited in an autosomal dominant pattern. The syndrome is characterized by growth of hamartomas and an increased risk of tumors of the thyroid (35%), breast (85%), kidney (34%), and endometrium (28%).¹ Common skin manifestations of Cowden syndrome include facial trichilemmomas, papillomatous papules, and acral keratoses.² Additional symptoms include macrocephaly and intellectual disability.¹ Cowden syndrome most commonly results from a variant in PTEN, a tumor suppressor, that is associated with uncontrolled cell division and the formation of hamartomas and cancerous tumors.³ Although there is no cure for the syndrome, recommended treatment involves a routine cancer screening schedule, as individuals are not only at an increased risk for cancer development but also tend to develop cancer at an earlier age. Screenings follow a timeline that involves annual thyroid ultrasonography beginning at age 18 years, colonoscopy beginning at age 35 years, kidney ultrasonography beginning at age 40 years, and annual skin checks. Additionally, female patients are recommended to undergo an annual mammogram beginning at ages 30 to 35 years and annual endometrial cancer ultrasonography beginning at ages 30 to 35 years.^4,5 Skin lesions associated with the syndrome are treated with surgical excision, chemical peels, and laser resurfacing.¹