Introduction Contrast-enhanced ultrasound is utilized in an increasing array of medical fields, including thoracic medicine. However, the technique is still relatively new and only sporadically mentioned in current guidelines and recommendations. The aim of this systematic review is to provide a literature overview and to critically appraise the current clinical applications of contrast-enhanced thoracic ultrasound (CETUS). Materials and Methods A systematic literature search using major electronic databases and in accordance with PRISMA guidelines was performed. Studies with a primary focus on CETUS of thoracic disorders compared to a standard reference test were included. The QUADAS-2 tool was used for quality assessment of the studies. Results The search identified 43 articles: 1 randomized controlled study, 6 non-randomized controlled studies, 16 non-randomized non-controlled studies, 5 case series, 10 single case reports, and 5 animal studies. The overall risk of bias was judged to be high. Diagnostic accuracy measurements of noninvasive applications of CETUS were only reported in a few studies and they were too dissimilar for meta-analysis. Six studies compared CETUS-guided versus ultrasound-guided transthoracic needle biopsy of thoracic masses. They individually reported a significant increase in diagnostic accuracy in favor of CETUS guidance but were too heterogeneous for meta-analysis. Conclusion The current literature on CETUS is overall heterogeneous with a few high evidence level studies, small study populations and a high risk of bias. CETUS-guided biopsy is the most frequent clinical application and increases diagnostic accuracy compared to ultrasound guidance by an average of 14.6 percentage points.