Adrenocortical carcinoma is a very rare tumor which can develop at any age in either sex, with an estimated incidence of 0.5-2 new cases per million per year. Only few cases of adrenocortical carcinoma are reported in the literature, probably because the differential diagnosis between malignant and benign mass is not easy. Surgery represents the treatment of choice for adrenocortical carcinoma and for its possible recidivisms. We report the case of a 36-year-old female patient affected by adrenocortical carcinoma, operated on in 1999 and re-operated on in 2002 for a liver metastasis of the same tumor, without any other treatment. This case may be interesting for its rarity as well as for the several difficulties of the diagnosis and the choice of treatment. In conclusion, the follow-up of these patients is mandatory due to the difficulties of diagnosis and curative surgery represents the best choice of treatment.