The coexistence of thyroid diseases with primary hyperparathyroidism (PHPT) can present a challenge in the clinical diagnosis and management for these patients. This study aims to determine the frequency of coexisting thyroid gland lesions in a consecutive series patients with PHPT, and to analyze the clinical features, diagnosis and treatment of these patients. Twenty-two cases of a total of 52 PHPT patients who had synchronous thyroid and parathyroid pathology were surgically managed in this study. Thirteen patients had ipsilateral thyroid nodules, and 9 patients had thyroid nodules in contralateral or bilateral side. Seven patients underwent direct parathyroidectomy and hemithyroidectomy via a mini-incision (about 3 cm), while other 15 procedures were converted to Kocher incision. Seventeen nodular goiter (32.7%), 2 thyroiditis (3.8%), 2 thyroid adenoma (3.8%) and 1 thyroid carcinoma (1.9%) coexisting with parathyroid adenoma were pathologically diagnosed. The sensitivity of preoperative ultrasonography (US) and methoxy-isobutyl-isonitrile (MIBI) scintigraphy for parathyroid lesions was 63.6% and 85.7%; and the overall positive predictive values for MIBI and US were 100% and 95.5% respectively. A high incidence of thyroid diseases that coexisted with PHPT in literatures was briefly reviewed. Our study illustrated the need for clinical awareness of concomitant PHPT and thyroid disease. A combination of US, computed tomography (CT) and MIBI scintigraphy would be recommended for preoperative localization of enlarged parathyroid adenoma and for evaluation of thyroid lesions. Synchronous treatment of associated thyroid abnormalities is desirable, and open minimally invasive surgical approach with additional resection of isolated ipsilateral thyroid nodules is possible in some of these patients.