Isosexual precocious puberty in girls is not uncommon, but its association with craniopharyngioma and growth hormone deficiency is rarely reported. We present a patient with this combination. An 8-year-old girl developed breasts and then had menarche at 7 9/12 years old. Growth hormone deficiency was suspected due to inappropriate height and growth velocity in association with idiopathic precocity and a poor predicted adult height of 138.8 cm. Growth hormone deficiency was confirmed by clonidine and insulin stimulation tests. Intracranial lesion was suspected due to precocity associated with GH deficiency. MRI of the sella's region revealed a 1 cm mass in the hypothalamus. After surgical resection, pathology of the tumor disclosed a craniopharyngioma which has rarely been reported to cause precocious puberty. The precocious puberty regressed after surgery. Growth hormone deficiency persisted and GH therapy was given to improve growth. The growth rate of patients with both growth hormone deficiency and precocious puberty may be maintained within the normal prepubertal range by the effect of sex steroid. We suggest that in patients with central type precocity in association with an inappropriate growth status, physicians should investigate the underlying intracranial lesion, and the possibility of growth hormone deficiency.