ABSTRACTBACKGROUND: Progressive pseudorheumatoid dysplasia (PPD) is a rare spondylo-epi-metaphyseal dysplasia (SEMD). It can be confused with juvenile idiopathic arthritis (JIA), both clinically andradiologically. Early detection and diagnosis of PPD are important in helping to relieve the pain and disability associated with this disease and in avoiding unnecessary investigations and anti-rheumaticinterventions.CASE DETAILS: We report the case of a 15-year-old girl with PPD who was misdiagnosed with JIA.CONCLUSION: In conclusion, PPD is a rare SEMD and can be confused with JIA, both clinically and radiologically. Early detection and diagnosis of PPD are important in helping to relieve the pain anddisability associated with this disease and in avoiding unnecessary investigations and anti-rheumatic interventions.KEYWORDS: Progressive pseudorheumatoid dysplasia, juvenile idiopathic arthritis, spondylo-epi-metaphyseal dysplasiaDOI: http://dx.doi.org/10.4314/ejhs.v26i4.12