Silicosis, an occupational lung disease that can be prevented, is still a significant public health concern in many countries, despite its considerably decreased incidence over the years. The latency period for silicosis ranges from a few years to several decades, depending on the duration and intensity of exposure to silica dust. The complex pathogenic mechanisms of the disease are not fully understood, but it is known to be characterized by inflammation, the formation of silicotic nodules, and progressive and irreversible fibrosis. The aim of this paper was to present the current sources of exposure to silica dust and summarize the updates on risk factors (e.g., socioeconomic status, genetic susceptibility) and sex differences, silico-tuberculosis, prognostic markers including 16-kDa Clara cell secretory protein, antifibrotic treatment, and other therapeutic possibilities with promising results. There are no effective treatment options for silicosis, and prevention remains the primary tool to significantly reduce the risk of disease. There are promising new treatments under investigation including antifibrotic, cellular, and immunomodulatory therapies, but further research is needed to demonstrate the efficacy and safety of these therapies in adequately powered clinical trials.