Background: Osteochondritis dissecans (OCD) of the capitellum is a painful condition, which often affects young throwing athletes. Our current understanding regarding the etiology, risks factors, diagnosis, and efficacy of the available treatment options has expanded over recent years, however remains suboptimal. Recent data on patient-reported outcomes following osteochondral autograft transplantation (OAT) for the treatment of large osteochondral lesions of the capitellum have been promising but limited. This review seeks to critically analyze and summarize the available literature on the etiology, diagnosis, and reported outcomes associated with OCD of the capitellum and the use of OAT for its treatment. Methods: A comprehensive literature search was conducted. Unique and customized search strategies were formulated in PubMed, Embase, Scopus, Web of Science, and CENTRAL. Combinations of keywords and controlled vocabulary terms were utilized in order to cast a broad net. Relevant clinical, biomechanical, anatomic and imaging studies were reviewed along with recent review articles, and case series. Results: Forty-three articles from our initial literature search were found to be relevant for this review. The majority of these articles were either review articles, clinical studies, anatomic or imaging studies or biomechanical studies. Conclusions: Current evidence suggests that OAT may lead to better and more consistent outcomes than previously described methods for treating large OCD lesions of the capitellum.