Unicuspid aortic valve disease (UAVD) is a frequent and long-lasting challenge for adult congenital heart disease centers. UAVD patients become usually symptomatic in their twenties or thirties and require a surgical treatment plan which should respect their complete lifespan combined with an adequate quality of life. Unfortunately, all current surgical strategies of congenital aortic valve disease bear some important limitations: (I) Aortic valve replacement using bioprosthetic valves is associated with early structural degeneration and leads frequently to re-operations. (II) Mechanical valves are commonly associated with lifelong risk of severe bleeding due to oral anticoagulation. (III) Using a pulmonary autograft (i.e., Ross procedure) for aortic valve replacement is associated with excellent long-term results in non-elderly patients. However, failure of pulmonary autograft or pulmonary homograft may require re-operations. (IV) Aortic valve repair or Ozaki procedure is only performed in a few heart centers worldwide and is associated with a limited reproducibility and early patch degeneration, suture dehiscence or increased risk of endocarditis. In contrast to degenerative tricuspid aortic valve disease, UAVD remains relatively understudied and reports on UAVD treatment are rare and usually limited to retrospective single-center observations. For this review, we searched PubMed for papers in the English language by using the search words unicuspid aortic valve, congenital aortic valve, Ross procedure, Ozaki procedure, aortic valve repair, mechanical/bioprosthetic aortic replacement, homograft. We read the abstracts of relevant titles to confirm their relevance, and the full papers were then extracted. References from extracted papers were checked for additional relevant reports. This review summarizes current surgical treatment strategies for UAVD including aortic valve replacement using bioprosthetic or mechanical valves, homografts, pulmonary autografts (i.e., Ross procedure) and aortic valve repair techniques for UAV. Furthermore, Ozaki procedure will be discussed.