SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a unique condition. Its rarity and variable presentation consummate its place within an elite group of elusory diagnosis. As a result, the diagnosis of SAPHO syndrome is often delayed by many years and is associated with increased medical cost, human suffering and worsened outcome. We present an atypical presentation of the SAPHO syndrome exhibiting an insidious onset of left hip pain with proximal femoral diaphyseal sterile osteitis along with severe weight loss that remained undiagnosed for over 5 years. We searched PubMed using SAPHO syndrome, unique SAPHO, SAPHO case study, SAPHO, CMRO and diaphysis as keywords for our literature review. The purpose of this case report is to emphasize a need for vigilance, timely recognition and prompt treatment. We further recommend aggressive early therapy for swift symptom resolution and improved medical outcome.