INTRODUCTION: Submandibular gland sarcoidosis is rare and little is known about its clinical presentation besides the usual neck swelling. The aim of the study was to extract clinical knowledge on submandibular sarcoidosis from the literature. METHODS: A systematic review was performed using a search in Medline with the key-words "sarcoidosis," "submandibular," "submaxillary." RESULTS: Forty-six articles fitting the search criteria were found, whereas 31 had to be excluded because they did not report submandibular gland sarcoidosis. Twenty cases of submandibular gland sarcoidosis were considered suitable for analysis. Almost all reported cases concerned female patients. In some cases submandibular gland's swelling is the first and only manifestation of the disease. CONCLUSION: Sarcoidosis should be considered in the differential diagnosis of all progressive and painless swellings of the submandibular gland, especially in women. Rarely, it may be the first manifestation of the disease.