This case reports a 21-year-old, homosexual African-American male who presented to our facility with a two-week history of progressive proximal muscle weakness. Quadriceps muscle biopsy showed a diagnosis of Nemaline Rod Myopathy, the presenting disease of his HIV infection. A review of the literature shows 13 prior case reports of similar disease process, often as the presenting symptom of the HIV disease. Anecdotal reports of effective treatment regimens include steroids and intravenous immune globulin; our patient had a profound response to high-dose steroids. This case report discusses this rare presentation of HIV in hopes to increase awareness amongst clinicians as the incidence and prevalence of HIV increases.