Thyrotropin-secreting pituitary tumors (TSH-omas) are a rare cause of hyperthyroidism and account for less than 1% of all pituitary adenomas. It is however noteworthy that the number of reported cases tripled in the last years as a consequence of the routine use of ultrasensitive immunometric assays for measuring TSH levels. Contrary to previous RIAs, ultrasensitive TSH assays allow a clear distinction between patients with suppressed and those with non-suppressed circulating TSH concentrations, i.e. between patients with primary hyperthyroidism (Graves' disease or toxic nodular goiter) and those with central hyperthyroidism (TSH-oma or pituitary resistance to thyroid hormone action). Failure to recognize the presence of a TSH-oma may result in dramatic consequences, such as improper thyroid ablation that may cause the pituitary tumor volume to further expand. The medical treatment of TSH-omas mainly rests on the administration of somatostatin analogs, such as octreotide and lanreotide. In fact, administration of dopamine agonists failed to persistently block TSH secretion in almost all patients and caused tumor shrinkage only in those with combined hypersecretion of TSH and PRL. On the contrary, somatostatin analogs were effective in reducing TSH and alpha-subunit secretion in more than 90% of cases with consequent normalization of FT4 and FT3 levels and restoration of the euthyroid state in the majority of them. In about one third of patients, a clear shrinkage of tumor mass and vision improvement could be demonstrated. Tachyphylaxis, cholelithiasis and carbohydrate intolerance occurred in a minority of treated patients. Whether somatostatin analog treatment may be an alternative to surgery and/or irradiation in patients with TSH-oma remains to be established. Nonetheless, the long-acting somatostatin preparations represent a useful tool for long-term treatment of such a rare pituitary tumors.