Anterior cervical hypertrichosis (ACH), or “hairy throat,” is a rare form of localized hypertrichosis that refers to the presence of a tuft of terminal hair on the anterior neck. Only 40 cases of ACH have been reported in the literature. Although it is usually an isolated finding, it may be associated with systemic disorders such as neurologic abnormalities (peripheral neuropathy, developmental delay, mental retardation), ophthalmologic disorders (optic atrophy, chorioretinal changes), hallux valgus, and dorsal hypertrichosis. Thus it is strongly advised to take a thorough family history and to perform clinical examinations and investigations (neurologic and ophthalmologic examination, electromyography, X-ray of the feet) in all patients with ACH to exclude possible associated abnormalities. We report the case of a 7-year-old Italian girl who presented with this condition as an isolated finding.