Introduction: Hereditary Transthyretin Cardiac Amyloidosis (hATTR-CA) of the Valine 122 Isoleucine (Val122Ile) variant is prevalent among 3.4% African Americans. More commonly diagnosed in men, there is limited data on sex differences in clinical characteristics and outcomes in patients with Val122Ile hATTR-CA. Methods: Retrospective chart review identified a total of 90 patients with a genetic confirmation of Val122Ile hATTR-CA and either positive technetium pyrophosphate (PYP) scanning or endomyocardial biopsy. Cohorts were divided based on sex and clinical and demographic data was collected from time of diagnosis. Results: Among our cohort, 64 (71%) were males and 26 (28%) were females. All were African Americans. Women were significantly older at the time of diagnosis compared to men, 76 ± 6 vs 71 ± 7, p=0.001. New York Heart Association (NYHA) Functional Classification scores were similar between sexes with the majority presenting with NYHA class II and III symptoms. Men had significantly higher rates of coronary artery disease compared to women, 17 (26.6%) vs 2 (7.7%), p=0.047, but all other comorbidities and extra-cardiac manifestations were similar. Troponin I, BNP, eGFR, and prealbumin did not differ significantly between sexes. Electrocardiographic variables, arrhythmia rates, and cardiac implantable electronic distribution were also similar between gender. However, several echocardiographic variables at diagnosis significantly differed: left ventricular ejection fraction (LVEF, %) was lower in men compared to women (41 ±17 vs 33 ± 15, p=0.024) whereas left ventricular end-diastolic diameter (LVeDd, mm) and mass index (LVMI, g/m2) were both higher in men (44.3 ± 6.7 vs 38.4 7.6, p = 0.001 and 168 ± 63 vs 131 ± 38, p = 0.009 respectively). There was no difference in time to diagnosis or survival between men and women in our cohort. Conclusions: Consistent with prior data, women with Va1122Ile hATTR-CA were significantly older with higher LVEF at diagnosis when compared to men. Despite this, women were found to have similar NYHA classification, extra-cardiac manifestation rates, biomarkers, and survival. These findings support a delayed, but equally infiltrative phenotype that should be considered in women with preserved heart failure.