Endocrine pancreatic tumors are rare neoplasms consisting of multipotent cells capable of secreting various bioactive substances causing characteristic clinical syndromes. Ovarian stromal hyperthecosis is characterized by varying degrees of luteinized stromal cell proliferation following sustained LH and/or hCG stimulation, clinically manifested by symptoms/signs of virilization resembling the polycystic ovary syndrome (PCOS). We report a case of ectopic bioactive LH production from a pancreatic endocrine tumor in a 33-yr-old woman with rapidly developing symptoms/signs of hyperandrogenism and markedly elevated serum androgen and LH levels leading to hyperthecosis and bilateral luteinized granulosa-theca cell tumors of the ovaries. Although the patient was initially thought to have either severe PCOS or an LH-secreting pituitary tumor, an LH producing pancreatic endocrine tumor bearing somatostatin receptors was demonstrated on scintigraphy with ¹¹¹In-octreotide and abdominal imaging. Symptoms and signs of hyperandrogenism resolved following the resection of the tumor. Immunohistochemistry, in situ hybridization and electron microscopy studies confirmed LH synthesis by the tumor cell. Although extremely rare, ectopic LH production from non-pituitary endocrine tumors should be considered in the differential diagnosis of hyperandrogenism particularly when associated with highly elevated serum LH levels.