Mixed connective tissue disease is a rare autoimmune rheumatological disorder of unclear etiology. Its association with silicosis is even rarer, but it is an intriguing relationship worthy of investigation. We present the case of a 45-year-old male patient who was diagnosed with silicosis after seven years of working in a quartz surface mine. Three years following his silicosis diagnosis, he developed rheumatological complaints and was subsequently diagnosed with mixed connective tissue disease. This case highlights the association of silicosis and mixed connective tissue disease. While rare, the available literature suggests that silica particles may contribute to the development of the disease.