Introduction Apical-variant hypertrophic cardiomyopathy (HCM) is a form of HCM in which hypertrophy is limited to the left-ventricular apex. Apical-variant HCM is rarely obstructive, though there is still an increased risk of atrial fibrillation, myocardial infarction, and sudden cardiac death (SCD). Apical-variant HCM has not previously been described in a patient with a prior diagnosis of dilated cardiomyopathy (DCM). Case A 53-year-old female with a history of hypertension and type-II diabetes mellitus presented with progressive dyspnea on exertion, fatigue and lower extremity swelling. Family history was significant for SCD of her mother at age 45. An initial transthoracic echocardiogram (TTE) showed DCM with an ejection fraction (EF) 15% and left ventricular end diastolic dimension (LVEDD) of 6.8 cm. The patient was placed on guideline-directed medical therapy (GDMT) and ultimately received cardiac resynchronization therapy with a defibrillator (CRT-D). One year later, EF was 25% with LVEDD 7.1 cm (Figure 1). A subsequent TTE two years later showed resolution of DCM with EF 55% and LVEDD of 5.1 cm. However, a new finding on this TTE was apparent: the apex demonstrated a spade-like shape on contraction consistent with apical-variant HCM (Figure 2). The patient underwent genetic testing but no genetic cause of either DCM or HCM was identified. Discussion HCM is caused by autosomal-dominant inheritance of a defective gene in sarcomere proteins. DCM and HCM are linked in that some etiologies of DCM are driven by sarcomeric protein abnormalities. DCM is also a rare phenotypical variant of HCM generally occurring very late in the disease and evolving from an initially hypertrophic myocardium. The opposite, in which DCM evolves into apical-variant HCM, to the best of our knowledge, has not been described in the literature. This relationship, as well as a family history of early SCD, suggest an inherited sarcomeric disorder manifesting as two discrete phenotypes within the same individual. This evolution may be due to treatment with GDMT, or it may be a rare expression of the disease's natural history. Only a fraction of cases of idiopathic DCM and HCM have an identifiable genetic cause and, thus, lacking an identified mutation does not preclude an inherited cardiomyopathy.