Avascular necrosis (AVN), a debilitating condition characterized by bone tissue death due to inadequate blood supply, can severely impact the hip joint, leading to pain, limited mobility, and joint dysfunction. The complex blood supply and mechanical stress on the hip make it particularly vulnerable to AVN. Early detection is challenging as AVN may remain asymptomatic initially, but as it progresses, it results in severe joint degeneration. This case report outlines the management of a 38-year-old male patient with a dermatomyositis history who presented with bilateral hip pain attributed to AVN. Radiological investigations diagnosed grade 2 AVN in the left hip and grade 3 AVN in the right hip. The patient underwent core decompression for the left hip to halt disease progression and total hip arthroplasty (THA) for the right hip to alleviate pain and restore function. A structured three-week rehabilitation program was tailored to each surgical procedure, with pre-and post-treatment assessments revealing notable improvements in pain relief, range of motion (ROM), and muscle strength. This case underscores the importance of early diagnosis, personalized surgical interventions, and comprehensive rehabilitation in managing AVN in dermatomyositis patients. Physiotherapy is vital pre- and post-operatively to enhance physical function, strength, and mobility. Rehabilitation also plays a crucial role in postoperative recovery, early mobilization, and functional restoration. The multifaceted approach employed in this case highlights the need for a comprehensive strategy when managing AVN in dermatomyositis patients, providing valuable insights for similar cases.