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Introduction: As the use of imaging increases, congenital organs malposition is detected more frequently. In order to clarify the specific anatomical complexity and features, three major categories, based on the position of the heart and the viscera relative to the midline, have been described situs solitus, situs inversus and situs ambiguous. Case Report: This is a case of a 59-year-old female presented to our hospital emergency room with dyspnea. Patient on clinical and radiological evaluation was diagnosed to have situs ambiguous with polysplenia and minor congenital heart malformations. Venous abnormalities, with double superior vena cava (SVC) and left inferior vena cava (IVC) were also present. Patient is currently asymptomatic and is on regular follow-up in our hospital, cardiology department. Conclusion: Developmental abnormalities unexpectedly found on imaging studies represent a radiological challenge. Careful analysis and understanding is mandatory, as anatomical miss arrangements can cause confusion in differential diagnosis and severe clinical implication during invasive procedures.

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