Craniopharyngiomas in children are usually associated with growth retardation and hypogonadism. A patient with a craniopharyngioma treated with radiotherapy, who later developed isosexual precocious puberty, is described. She was treated with cyproterone acetate with a good clinical response. We suggest that craniopharyngiomas may become a more frequent cause of precocious puberty now that conservative therapy has been advocated for the condition.