Sarcoidosis is an idiopathic multi-system granulomatous disease that frequently affects the lungs and the lymph nodes of young adults with a female predilection. While its diagnosis is based on clinical and radiological findings with exclusion of other more specific conditions, the demonstration of non-caseating granulomas on biopsy tissue is mandatory. Genetic, infectious, and environmental factors have been postulated as possible causes, but its etiology remains unknown. The head and neck region is affected in 10–15% of the cases, mostly presenting as swelling and xerostomia involving the salivary glands. Oral involvement is unusual, and lesions can be solitary or multiple, more often affecting the buccal mucosa, gingiva, lip, palate, and tongue. Patients are usually asymptomatic, and the lesions range from normal colored to reddish papules or nodules, but ulcerated lesions may be painful. Occasionally, drug usage may develop a drug-induced sarcoidosis-like reaction that is clinically and microscopically identical to sarcoidosis, but that typically improves or resolves after the drug discontinuation. Sarcoidosis treatment frequently comprises the use of corticosteroids, which may or may not be combined with different immunosuppressors or antimalarial drugs.