Fasioscapulohumeral muscular distrophia is a slow progressing sickness which shows heredity, starting during childhood and adolescence period and is characterized by muscle weakness especially around face. Anchilosing spondylitis is muscle and ligament illness effecting sacroiliac joint and lomber vertebrae. A 31 years old patient consulted due to having progressively increasing speaking and swallowing difficulties. It has been understood that, at the age of 18, there was weakness complaints spread all around the body, especially distinctive in shoulder and neck area. In the patient's two brothers, in whose mother muscular distrophy detected, it was determined that they had spread muscle weakness. The patient, in whose physical examination, both of the eyes uveitis symptom, fasioscapulohumeral type muscle atrophy was observed, had myopathic facial expression and shoulder, neck movements were limited and muscle strength was 4/5 in his neurologic examination. In laboratory checks, ESR:99 mm/h, CPK:146, EMG was consistent with myopathy. Right-side deltoid muscle biopsy was consistent with vasculitis and myositis. HLA B27(+). Radiological findings consistent with anchilosing spondylitis were detected. Anchilosing spondylitis and fasioscapulohumeral muscular distrophia togetherness, anchilosing spondylitis and myopathy togetherness were taken into consideration in the light of literature.