We report a case of Stickler syndrome associated with slipped capital femoral epiphysis. A 10-year-old male subsequently developed left thigh pain without any provoking cause. Three days after, when he swung a bat, marked left hip pain developed. Radiograph showed a slipped capital femoral epiphysis. At the age of 1 year, he underwent surgery due to cleft palate. The visual acuity was bilaterally decreased, and a diagnosis of bilateral retinal/vitreous degeneration had been made. Radiograph on the normal side showed coxa valga. According to clinical symptoms and past history, diagnosis of Stickler syndrome associated with slipped capital femoral epiphysis was made. On the day of the first visit, surgery was performed for slipped capital femoral epiphysis. No intentional reduction was performed. After gentle protective traction, in situ fixation was performed with 6.5 mm cannulated cancellous screw. After 5-month nonweight-bearing, weight-bearing was initiated, and full-weight bearing was achieved after 1 month. MRI 6 months after the operation showed no bone necrosis, and lateral radiography confirmed bone union.