A heterotopic salivary gland (HSG) in the lower neck is an unusual developmental anomaly rarely encountered by the dermatologist. Klimko and Horanyi (1) reported the first histologically documented case of HSG in the lower neck in 1958. To our knowledge, fewer than 100 such cases have been reported in the literature to date. The origin of HSG in the lower neck seems to be closely associated with the branchial apparatus. Because this condition is rarely encountered, dermatologists should be well informed about the clinical manifestations, development of various malignancies, and the differential diagnosis of HSG.