Background: Hydatid disease caused by Echinococcus granulosus commonly involves the liver followed by lungs. Cardiac involvement is a rare occurrence and presents a challenging scenario. Case presentation: Our case describes a middle-aged gentleman who presented to the emergency room with an episode of sudden loss of consciousness resulting from ventricular tachycardia. After successful cardiac resuscitation, the patient underwent imaging that showed a lesion compatible with hydatid cyst. Surgical treatment with pharmacologic coverage was provided which resulted in good clinical outcome. Conclusions: The case highlights rare occurrence of isolated cardiac hydatid disease presenting as cardiac arrhythmia in contrast to its common routine outpatient presentation involving the liver and lungs. Good knowledge of the unusual presentations and its epidemiology is essential to the proper management of such patients.