Objective:To summarize the clinical manifestations and color Doppler ultrasonography of benign lymphoepithelial disease (BLL) in lacrimal gland, so as to improve the diagnostic accuracy of lacrimal BLL.Methods:Clinical manifestations and color Doppler flow imaging (CDFI) features of lacrimal BLL in 16 patients (2 males and 14 females, with 31 lesions) who visited the Second Hospital of Hebei Medical University from November 2014 to August 2019 were retrospectively analyzed.Results:The performance for lesions in patients with duration less than 3 months was scattered in irregular low echo, lesion blood flow signals within the rich (Adler Ⅱ level), pathologic examination results showed more lymphocytes were seen in the lacrimal matrix, and the myoepithelium of the lacrimal duct grew to form the epithelium-myocutaneous island. The ultrasonic feasures in patients with course of 3-6 months were in multiple categories such as circular low echo, "honeycomb" change, CDFI showed lesions with a lot of blood flow signals (Adler Ⅲ level), the pathological examination results indicated that there were a large number of lymphocytes and epithelial-musculocutaneous islands in the lacrimal matrix, and the lymphocytes were significantly increased compared with patients with the course of disease less than 3 months. The ultrasound results in patients with a course of more than 6 months showed lesions in multiple categories such as circular low echo, and large low echo, greater than 3 mm in diameter, CDFI showed lesions within the same large amounts of blood flow signals (Adler Ⅲ level), the pathological examination results were consistent with the course of 3 to 6 months. One patient had positive tuberculin test and 11 had higher IgG4 than normal.The number of lymphocytes increased with the prolongation of disease course.Conclusions:With the expert knowledge of color Doppler ultrasonographic characteristics of lacrimal gland BLL and with the serum IgG4 level helps to improve the accuracy of ultrasonic diagnosis.