Coronary-cameral fistulae (CCF) are rare, frequently incidental findings uncommonly noted during routine coronary angiography. They are nearly always congenital and are sometimes associated with other cardiac malformations. They can also be acquired due to trauma or chronic inflammation. These fistulae most commonly originate from the right coronary artery. The site of termination is usually the right ventricle (RV) and rarely the left ventricle (LV). Though nearly always asymptomatic and clinically insignificant, depending on their size and pressure gradient between communicating sites and terminating area, CCF can lead to pulmonary hypertension, LV dysfunction, and myocardial infarction. We describe the case of a 55-year-old woman who presented with worsening dyspnea and lower extremity edema. Transthoracic echocardiography demonstrated an ejection fraction of 55% with an RV systolic pressure of 67 mmHg. Right heart catheterization was performed to formally diagnose pulmonary hypertension and left heart catheterization was performed concurrently. This demonstrated a fistula between the first obtuse marginal branch of the left circumflex artery to the LV cavity. In this report, the authors provide a brief review of the presentation, diagnosis, complications, and management of CCF.