BACKGROUND Thyroid Hurthle cell neoplasm (THCN) is relatively rare. OBJECTIVE To describe the presentation, diagnostic approach and management of THCN in our institution. METHODS This was a retrospective chart review of all thyroid Hurthle cell neoplasms diagnosed at Aseer Central Hospital (ACH), Saudi Arabia during the period from October 1998 to April 2007. Data including clinical, cytopathologic, radiologic, histopathologic and surgical treatment were extracted for analysis. RESULTS Nine patients were diagnosed as THCN (eight females and one male). Their ages ranged from 24-49 years. Three cases were Hurthle cell carcinomas and six cases were Hurthle cell adenomas. Carcinomas presented as solitary nodules (two cases) and as multinodular goiter (one case). Adenomas presented as solitary nodules (two cases), as multinodular goiter (three cases) and as diffuse swelling (one case). Fine needle aspiration cytology (FNAC) was diagnostic for THCN in two cases of carcinoma that presented as solitary nodules and hence total thyroidectomy was performed. Total thyroidectomy was also done in one case of adenoma. Hemithyroidectomy was performed in two cases of adenoma in which FNAC showed benign lesion and in one case of carcinoma based on clinical and ultrasonographic impression of benign MNG in the involved lobe and inconclusive FNAC result. Subtotal thyroidectomy was performed in one case of adenoma. CONCLUSIONS Preoperative diagnosis and management of THCN is still a dilemma. Neither clinical nor FNAC findings can exclude carcinoma. Therefore a combination of clinical, radiological, FNAC and histopathological results should be used for a more definitive subtyping and proper management.