Noncompaction of ventricular myocardium (NVM), a relatively new diagnostic entity, is described as an arrest in the process of compaction of myocardial fibers, which results in a prominent trabecular network and deep intertrabecular recesses. Its coexistence with other cardiac anomalies like hypertrophic obstructive cardiomyopathy (HOCM) or polycystic kidney disease (PKD) had been reported in the past. We report the first case with all 3 different inherent conditions (NVM, HOCM, and PKD) manifesting in 1 patient. A 37-year-old man was referred for evaluation of a heart murmur. His medical history was positive for paroxysmal atrial fibrillation. Physical examination revealed a grade 3/6 systolic murmur loudest along the left sternal border accentuating on Valsalva maneuver. Echocardiography revealed HOCM. Cardiac magnetic resonance confirmed the presence of HOCM with the incidental finding of NVM and PKD. This case raises the possibility of genetic mutation common to these 3 clinical entities or 2 different gene mutations existing in the same individual.