Being the most common childhood tumor to involve the hypothalamus and pituitary gland, craniopharyngioma, a histologically benign tumor, is usually related to growth retardation and hypogonadism, The presentation of precocious puberty is very rare for a hypothalamic craniopharyngioma, Here, we report such a case. The female patient had presented with symptoms of menarche and breast development since she was 6 years old. Hormonal therapy with a gonadotropin-releasing hormone analogue was instituted to cease precocious puberty but was unsuccessful, Magnetic resonance imaging of the sella showed a l-cm tumor in the hypothalamic area, Through a pterional approach, the tumor was removed en bloc with endoscope-assisted microsurgery. After the operation, our patient's symptoms improved, so the hormone therapy was discontinued, This extremely unique association of craniopharyngioma and precocious puberty, the causes and mechanisms involved a I on g with the advantages of endoscope-assisted microsurgery are discussed. Copyright (C) 2001 S. Karger AG. Basel.