Anterior cervical hypertrichosis (ACH) refers to a patch of hair that is presented at the lower anterior aspect of the neck just above the sternal notch along the cervical midline. The condition occurs as an isolated defect or in association with other abnormalities. Eighteen out of the 28 ACH patients who were previously reported on in the English literature showed a familial inheritance pattern. Some of the reported cases were associated with other clinical symptoms such as peripheral sensory and motor neuropathy, developmental delay, retinal change and bony abnormality. Herein, we report on a case of 2 sisters and 1 brother who had familial anterior cervical hypertrichosis without any other abnormality.