Pituitary carcinoma is a extremely rare and is characterized by a very poor prognosis. Even if at diagnosis the presence of metastases is required to define a pituitary carcinoma, the lesion was almost invariably diagnosed first as a benign pituitary tumor, that after a variable period of latency, ranging from few months to many years, changed its natural course to an aggressive pituitary tumor poorly responsive to therapy. Recent studies have partially clarified its molecular pathogenesis and found possible markers of aggressiveness in order to make an earlier diagnosis, when still treatment could improve their prognosis. Most pituitary carcinomas are functioning, and ACTH- and PRL-secreting carcinomas are the most frequent. Treatment includes surgery, radiotherapy, medical therapy and chemotherapy, but the poor results with current therapies should prompt all investigators to better understand its pathogenesis and searching new molecular targets for treatments.