Mammary fistulas occurring in males are extremely rare, the first being described in 1974 by Tedeschi et al. (1). Our detailed review of the world literature has revealed a total of just 20 cases of mammary fistulas in males; a total which includes three cases of mammary fistulas in infants and three cases associated with human immunodeficiency virus (HIV) infection. Mammary fistulas develop between the lactiferous ducts and areolar skin. Altered duct anatomy and microarchitecture may predispose a patient to mammary fistulas. This discussion focuses on mammary fistulas in males where smoking is a likely etiologic factor, and therefore examines a clinical rarity with just 14 reported cases in the literature. The treatment of mammary fistulas in males is the same as for females once the diagnosis has been made.