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Marfan syndrome is a spectrum of disorders caused by a genetic defect involving connective tissue and is heritable by the autosomal dominant mode of inheritance. Atlantooccipital assimilation is a partial or complete fusion of the atlas and the occiput base congenitally. Although primarily asymptomatic, some patients with atlantooccipital assimilation may present with neurological issues, including myelopathy. Here, we are discussing a case of an 18-year-old male who presented with bilateral paraesthesia, tingling and neck pain which, upon investigations, turned out to be a case of atlantooccipital assimilation along with basilar invagination with spinal cord compression. The patient also had marfanoid features like tall stature, reduced upper to lower segment ratio, and increased arm span to height with positive wrist and thumb signs. As myelopathy had already developed, the patient was treated surgically rather than with medical management with a favorable outcome.

L-looped transposition of great arteries (L-TGA) is an extremely rare heart condition. It is associated with physiologically corrected transposition of great vessels, leading to the normal return of deoxygenated systemic venous blood to the heart and transport of oxygenated pulmonary venous blood to the main systemic circulation. Anatomic discordancy and anomalous coronary artery distribution predispose the right ventricle to excessive workload and eventual heart failure. This mandates anatomic correction or proper medical management of heart failure. Herein, we present a case of a 14-year-old girl who presented to our cardiology consults with a false impression of pre-made clinical diagnosis of tetralogy of Fallot. She had increasing dyspnea and cyanosis on presentation. Striking marfanoid habitus and unusual echocardiographic findings of tripartite geometry of heart with parallel and discordant positioning of atria, ventricles, and great arteries led us to cardiac computed tomography which confirmed the diagnosis of L-TGA. Our patient also had associated patent ductus arteriosus, dextrocardia, ventricular septal defect (VSD), and pulmonary atresia. Due to the complex nature of heart disease and unavailability of resources, she was treated with a comprehensive heart failure protocol and followed up clinically and radiologically at regular intervals and showed massive improvement. This is the first-ever documented case of L-TGA with complex shunting and marfanoid habitus.