Background <p>Double-inlet right ventricle is an extremely rare and complex cardiac malformation characterized by the opening of both atria into the right ventricle and is usually associated with other major cardiac malformations. In this case report, we have discussed an isolated case of double-inlet right ventricle without other malformations and conducted a literature review to compare our case, which is the third in the medical literature, with previously published papers on double-inlet right ventricle.</p> Case presentation <p>A 2-month-old Syrian male infant was admitted to the cardiology department due to symptoms of central cyanosis and failure to thrive. Echocardiography revealed double-inlet right ventricle with a hypoplastic left ventricle from which the hypoplastic aorta emerged, while the pulmonary trunk emerged from the dilated right ventricle. The child was sent abroad for the Norwood procedure.</p> Conclusion <p>Although double-inlet right ventricle is a very rare malformation, it can be encountered in clinical practice and should be taken into consideration by practitioners. Clinical researchers should document and publish cases of double-inlet right ventricle diagnosed to draw attention to this malformation and study it in greater depth.</p>