Uremic tumoral calcinosis (UTC) is a severe complication in patients undergoing maintenance hemodialysis.It is characterized by massive ectopic periarticular deposition of calcium–phosphorus compounds, especially around the weightbearing joints.We herein describe a 39-year-old uremic female on long-term continuous ambulatory hemodialysis for 8 years who developed UTC in the right gluteal region,featuring severe soreness and restriction of her right hip joint. She underwent incomplete surgical excision, showing typical tumoral calcinosis on histology, resolved the clinical symptoms.In addition, we conducted a pertinent literature search on PubMed,EBSCO, Ovid LWW and Springer database on the basis of the keywords “tumoral calcinosis”, without any time restriction, than we identified additional articles by reviewing the citations of published articles on the topic.We present a concise review on the etiology, pathogenesis, histological characteristics, diagnosis, available treatment options and overall systemic management of this particular disease based on the literature search.