Nevoid basal cell carcinoma syndrome (NBCCS) has been known to coincide with different forms of other neoplasias; however, parathyroid adenoma in this syndrome has not previously been described. The authors report a case of such association in a 50-year old white woman. The adenoma was verified before operation by biochemical, isotopic and cytologic methods and later, after the excision of adenoma, histologically.

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