Fibromuscular dysplasia (FMD) is defined by focal narrowing of small and medium-sized arteries due to an idiopathic, noninflammatory, nonatherosclerotic vascular disease. The population-based prevalence of FMD remains unknown. Using the National Inpatient Sample database, we evaluated the prevalence, clinical presentation, mortality, and associated conditions of FMD from January 1, 2004, to September 30, 2015. Among 2,420 patients who presented with FMD, 2,086 (86.20%) of patients were female. The mean age was 55.18 ± 18.99 years in men and 63.37 ± 17.10 years in women. FMD patients most commonly presented with hypertension (67.3%), transient ischemic attack (3.7%), headache (2.1%), dizziness (1.1%), abdominal pain (0.6%), or hematuria (0.3%). In-hospital mortality of FMD patients was 0.74%. In conclusion, FMD is a rare condition with low in-hospital mortality that may be considered among female patients presenting with hypertension.