<p>Ivemark syndrome, a classification of heterotaxy syndrome, is an extremely uncommon congenital condition. An aberrant configuration of the thoracoabdominal organs across the body’s left–right axis is known as heterotaxy, and it is always linked to either left or right atrial isomerism. It is distinguished by the underdevelopment or absence of the spleen, cardiac abnormalities, and an irregular configuration. This comprehensive review is put forward with the objective of overcoming the challenges faced in treating and managing the clinical signs and symptoms of Ivemark syndrome. This is proposed to create an awareness of Ivemark syndrome, a cardiac-related disorder, and its complications and how to manage the clinical aspect of treating this condition. ‘Heterotaxy syndrome’ and ‘Ivemark syndrome’ were used as key terms in a search by using Pubmed, Embase, and Scopus as the reference sources with clinical inquiries. Clinical trials, meta-analyses, observational studies, and opinions were covered in the seek method. The search was limited to English. The current article was put together using the data that was found during the aforementioned search. Since Ivemark syndrome occurrences are primarily sporadic, the underlying cause of the disorder is yet unknown. Bradycardia is the primary warning symptom. Sonography can be used to diagnose pregnancies. This condition is typically diagnosed in the newborn stage. Physicians need to get versed in this condition and how to treat it. The purpose of this article was to present an overview of the complications and treatment options for Ivemark syndrome.</p>