ABSTRACT
Adrenocortical carcinoma is a relatively rare malignant tumour, which progreses rapidly within a few months. in this report an adrenocortical carcinoma case with atypical clinical progression is presented. Although she had clinical findings at the 2nd year of age she was recognized at the age of 11 years as presenting heterosexual precocious puberty accompanied by an increase in the steroid precursors in the form of 21-hydroxylase enzyme deficiency.