Stickler syndrome is an autosomal dominant connective tissue disorder with a prevalence similar to that of Marfan syndrome. No previous study has examined hip pain or abnormalities in a large series of patients with Stickler syndrome. The purpose of this study was to describe hip abnormalities and their correlation with age and chronic hip pain in a cohort of 51 patients followed at the National Institutes of Health. Ten percent of patients had protrusio acetabuli, 21% coxa valga, and 34% of adults had hip osteoarthritis. Sixty-three percent of all patients and 79% of adults had chronic hip pain. In addition, 16% of adult patients had a history of femoral head failure during youth. Arthritic changes and adult age were associated with hip pain. In summary, hip abnormalities are commonly observed in Stickler syndrome. Young patients require careful evaluation of hip pain, and regular screening of children with Stickler syndrome may be indicated for early detection of hip complications.