Background <p>Sinus of Valsalva aneurysm is a relatively rare disease accompanied by several severe complications. Patients with an unruptured sinus of Valsalva aneurysm are usually asymptomatic or present with symptoms of dyspnea, easy fatigability, palpitations, or chest pain, whereas a ruptured sinus of Valsalva aneurysm may cause severe symptoms including heart failure, myocardial ischemia, or even cardiac arrest. Misdiagnosis is common in clinical practice owing to the rarity of sinus of Valsalva aneurysm and its highly variable, often atypical clinical presentations, which can mimic more common conditions.</p> Case presentation <p>We present a rare and interesting case of a patient with a ruptured sinus of Valsalva aneurysm, a 33-year-old Chinese man who was misdiagnosed owing to a series of atypical symptoms, including eyelid and facial edema and dyspepsia. Fortunately, the patient was finally diagnosed with ruptured sinus of Valsalva aneurysm, and transthoracic echocardiography showed the noncoronary sinus of Valsalva aneurysm, which ruptured into the right atrium, accompanied by cardiac enlargement and pericardial effusion.</p> Conclusion <p>This case highlights the importance of obtaining a complete history and a standardized physical examination, and emphasizes the need to consider ruptured sinus of Valsalva aneurysm in young patients presenting with sudden heart failure without a prior cardiac history, and that transthoracic echocardiography is important in providing an accurate and rapid sinus of Valsalva aneurysm diagnosis.</p>