Objective To investigate the optimal diagnosis and treatment strategy of occult carcinoma of the thyroid (OCT) with neck lymph node metastasis as the first symptom. Method In order to discuss the optimal diagnosis and treatment strategy of OCT with neck lymph node metastasis as the first symptom, we collected 35 cases and analyzed their characteristics, diagnostic methods, operative schemes, metastasis situation, and death situation. Results Of the 35 cases, 28 cases went to hospital because of swollen lymph nodes, and other 7 cases were discovered by color Doppler ultrasound in medical examination. Thyroid nodules were found by color Doppler ultrasound in 32 cases, 3 cases were found no thyroid nodule. Lymph node of 23 cases were determined by ultrasound-guided fine-needle aspiration biopsy (US-FNAB), and 16 cases (69.56%) were diagnosed as metastasis of thyroid carcinoma or suspicious metastasis by US-FNAB. Thyroid biopsy were done in 21 cases, and 11 cases (52.38%) were diagnosed as thyroid carcinoma or suspicious thyroid carcinoma by fine needle aspiration biopsy. Of the 35 cases, 19 cases were performed total thyroidectomy and functional neck lymph node dissection, 11 cases were performed resection of unilateral thyroid and isthmus and regional neck lymph node dissection, 5 cases were performed nonstandard operations. All cases were followed up for 3–10 years after operation, and the median time was 7-year. During follow up period, 10 cases suffered from reccurrence. Among them, 3 cases reoccurred in the nonstandard operation group, 5 cases reoccurred in resection of unilateral thyroid and isthmus and regional neck lymph node dissection group, 3 cases reoccurred in total thyroidectomy and functional neck lymph node dissection group. There were 3 cases died. Among them, there was 1 case in each group of nonstandard operation group, resection of unilateral thyroid and isthmus and regional neck lymph node dissection group, and total thyroidectomy and functional neck lymph node dissection group. The recurrence rate of total thyroidectomy and functional neck lymph node dissection group was markedly lower than those of resection of unilateral thyroid and isthmus and regional neck dissection group (χ2=4.751,P<0.05) and nonstandard operation group (χ2=5.874,P<0.05). While there was no significance difference of the recurrence rate between the resection of unilateral thyroid and isthmus and regional neck dissection group and nonstandard operation group (χ2=0.291,P>0.05). There was no significance difference in the mortality among the three groups (P>0.05). Conclusion US-FNAB and intraoperation rapid frozen pathological section are important methods for diagnosis of OCT with neck lymph node metastasis as the first symptom, and standard operation is an principal treatment method for it.