Background: The purpose of this paper is to review the MRI features and treatment for idiopathic chondrolysis of the hip (ICH) in patients aged 18 years or less. Methods: We included studies published in English up to August 2021. We accessed major electronic bibliographic databases on ICH that described MRI features, treatment, or both. We used the Joanna Briggs Institute (JBI) Critical appraisal checklist for case reports for Risk of bias assessment.Results: We pooled 136 hips (125 participants) from 35 studies with 11.6 ± 3.4 years mean age. We had 46, 8, and 106 hips to assess ICH's MRI, pharmacological, and operative interventions. Geometric marrow edema (GME) (P < 0.01), diffuse marrow edema (DME) (P < 0.05), diffuse cartilage loss (DCL) (P < 0.05), and joint effusion (P < 0.05), were significantly associated with time in first MRI reviews. GME (P < 0.01) and focal cartilage loss (FCL) (P < 0.01) decreased significantly between two MRI reviews at median time of 1.75 (IQR 0.93-4.25) and 12.5 (IQR 3.75-19.5) months. Diffuse cartilage loss (P < 0.01) and degenerative changes (P < 0.01) increased significantly between the two MRI reviews. Etanercept, Methotrexate, and Botulinum Neurotoxin A drugs were used by 3, 3, and 1 report to treat ICH. Capsulectomies, total hip arthroplasty, arthrodiastasis, arthrodesis, arthroscopy operations treated 45, 18, 5, 5, and 2 hips. Discussion: GME may be the most specific and early MRI feature in diagnosing ICH. GME and DME show an inverse relationship over time. So, it is with FCL and DCL. Despite reports on the efficacy of biologics, immunomodulators, and operations, early and late ICH management remains controversial due to poor quality studies.