Background: Dextrocardia is a rare congenital anomaly, seen in about 1 in 12,000 births, characterized by abnormal cardiac position and thought to result from early cardiac developmental disturbances. This report presents the unusual coexistence of dextrocardia and a posterior fossa arachnoid cyst, a combination seldom described. Case summary: A 25-year-old woman presented with exertional dyspnea, orthopnea, paroxysmal nocturnal dyspnea, fatigue, and bilateral peripheral edema. Examination revealed elevated jugular venous pressure, tachycardia, a systolic murmur, ascites, and lower limb edema. Chest X-ray showed dextrocardia and cardiomegaly, while brain CT identified an arachnoid cyst. Laboratory tests demonstrated mild anemia with normal renal function and serum proteins. She was diagnosed with congestive heart failure, started on medical therapy, and chose outpatient follow-up for the cyst without further intervention. Discussion: The coexistence of dextrocardia and an arachnoid cyst suggests a potential genetic link and supports comprehensive genetic evaluation in patients with congenital anomalies. Recognizing such associations may improve understanding of underlying developmental mechanisms and help refine diagnostic and management strategies.