Developmental dysplasia of the hip (DDH) in adolescents and young adults can cause notable pain and dysfunction and is a leading cause of progressive hip osteoarthritis in affected patients. Recognition of the clinical symptoms and radiographic presentation of DDH in adolescents and young adults are paramount for early management. Plain radiographs are critical for making proper diagnosis, whereas three-dimensional imaging including MRI and/or CT detects intra-articular pathology and better characterizes hip morphology. Management of early, symptomatic DDH includes nonsurgical modalities and open joint preservation techniques. Arthroscopic management can be used as an adjunct for symptomatic treatment and for addressing intra-articular pathology, but it alone does not correct the underlying osseous dysplasia and associated instability. The periacetabular osteotomy has become the mainstay of efforts to redirect the acetabulum and preserve the articular integrity of the hip; however, the proximal femur is also a potential source of pathology that should be considered. Open hip procedures are technically demanding yet provide the opportunity for pain relief, improved function, and preservation of the hip joint.