Background: In addition to the typical form resembling the classical Japanese octopus trap, atypical variants of Takotsubo cardiomyopathy (TTC) sparing the left ventricular apex have emerged over the years. The aim of this systematic review is to provide a comprehensive overview of the cardiac nuclear imaging findings in atypical variants. Methods: This systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The literature research was carried out online on the Pubmed, Scopus, Central (Cochrane Library), and Web Of Science databases. Results: A total of 14 articles were ultimately selected. Myocardial perfusion scintigraphy was performed in nine studies, followed by 123I-mIBG scintigraphy, 123I-BMIPP scintigraphy, and 18F-FDG PET. In seven cases, a single cardiac nuclear imaging technique was performed, while in the remaining five and two cases, two and three different imaging modalities were, respectively, used. The most common atypical variant of our selection was the midventricular form, followed by reverse/inverted/basal TTC, with only a single case reported of a focal pattern. Conclusions: As the reason why TTC variants occur is still not clear, a deeper understanding of the current knowledge could be the basis for providing more insights into this fascinating disorder and its uncommon manifestations.