<h3>Introduction</h3> Histiocytoid cardiomyopathy (HC) is a rare form of cardiomyopathy associated with ventricular arrhythmias and sudden death in infancy. Most cases are diagnosed at autopsy or, occasionally, after heart explant at heart transplantation (HT). We report the case of a toddler with recalcitrant arrhythmias despite multiple medications for whom HC was not suspected. The patient underwent HT, after which biopsy of the explanted heart revealed HC. This case highlights the potential value of a pre-transplant diagnosis of this rare disease, especially in age groups with anticipated long waitlist times. <h3>Case Report</h3> A 16-month old female patient was admitted to the cardiac intensive care unit for Wolf-Parkinson White syndrome (WPW) and recalcitrant ventricular tachycardia (VT) with left ventricular noncompaction (LVNC). She underwent ICD placement, bilateral sympathectomies, and transitioned to oral antiarrhythmic agents with adequate rhythm control. She required readmission for multiple AICD discharges for VT and VF. Despite maximizing antiarrhythmic therapy, she had increasing episodes of VT and was ultimately listed for HT (status 1A by exception). She remained on the waitlist for 37 days prior undergoing HT. Biopsy of her explanted heart showed subendocardial fibrosis, a diffuse nodular thickening of the subendocardium with a proliferation of clear cuboidal/polygonal cells that have granular cytoplasm and bland round nuclei, and stained positive for MYOD1, findings diagnostic for HC in addition to LVNC (Figure 1). <h3>Summary</h3> HC, particularly alongside LVNC, is a rare condition that requires endomyocardial tissue for diagnosis. Pre-HT diagnosis is not described. In this case, pre-transplant listing likely would have prompted earlier listing for HT. Despite the risks associated with catheterization and biopsy in patients with cardiomyopathy and arrhythmia, the pursuit of a pre-HT diagnosis may be warranted to affect patient management and justify status 1A exception requests.