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Abstract The term 'angiokeratoma' includes a wide range of dermatological conditions of hyperkeratotic vascular disorders with a similar histologic combination of hyperkeratosis and superficial dermal vascular ectasia. Angiokeratomas can be classified into localized and systemic forms. Angiokeratoma of Fordyce (AKF) is a localized form of angiokeratoma, clinically characterized by 1- to 6-mm, black, blue, or dark red, dome-shaped papules located on the scrotum, shaft of penis, labia majora, clitoris, inner thigh, and lower abdomen. We describe herein a case of giant angiokeratoma of Fordyce on shaft of the penis in an elderly man, clinically masquerading as keratoacanthoma.