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Ectopic hormonal syndrome, the most common cause of paraneoplastic syndrome, is due to an inappropriate secretion of peptide hormones by neoplasms derived from non-endocrine tissue, often discovered even by an occult neoplasm. The diagnosis of ectopic hormonal syndrome is established based on the following criteria;1. abnormal clinical signs and symptoms and/or inappropriate hormonal secretion associated with tumors;2. the abnormal endocrine syndrome and hormone secretion are reversed by tumor resection, and relapsed after tumor recurrence; 3. an arteriovenous gradient for the hormone across the tumor; and 4. the hormone can be detected in the tumor tissue. The treatment of choice for ectopic hormonal syndrome is directed at the primary tumor by surgical resection, radiotherapy, or chemotherapy, and the palliative treatment to control excess hormone secretion is effective in alleviation of symptoms.