Hypertrichosis lanuginosa acquisita (HLA) is an unusual condition which is characterized by subtle and progressive development of multiple, long, thin, unmedullated hairs ("lanugo hairs") distributed preferentially on the face. Most cases are associated with malignant tumors or non-malignant condition such as porphyria cutanea tarda, AIDS, anorexia nervosa, thyrotoxicosis, or secondary to topical or systemic drugs (e.g. cyclosporine, phenytoin, diazoxide, minoxidil). We have recently experienced a rare case of hypertrichosis lanuginosa acquisita associated with autoimmune hepatitis. To our best knowledge, this is the first report of hypertrichosis lanuginosa acquisita associated with autoimmune hepatitis. Our observation expands the spectrum of diseases associated with this uncommon disorder.