Shone syndrome is a rare congenital cardiac abnormality; however, many of the cases remain undiagnosed until early and middle adulthood. Different imaging modalities are used to assess the related structural abnormalities. In this case study, we report a 32-year-old woman who was planning her first pregnancy. In light of her childhood heart problems, in addition to a history of extended penicillin prescriptions for several years, she was referred for complementary assessments. At the time of presentation, she was asymptomatic. Imaging results showed several structural obstructive left-sided lesions and pulmonary artery hypertension. Ultimately, the patient was diagnosed with congenital shone syndrome, which was initially misdiagnosed. Shone complex in our case was presented in its full form, which could be potentially fatal in case of pregnancy.