Tumoral calcinosis has long been a disputed clinical-pathological subject. It has been characterized by the deposition of calcium salt and hydroxyapatite in different periarticular soft tissue regions. It is most commonly seen in adults, and rarely seen in children. In this report, we present the case of a six-year-old girl referred to our institution for one year with a history of left hip pain and swelling. The patient underwent an open reduction of left hip and acetabuloplasty for developmental dysplasia of the hip (DDH) at the age of two years. Systematic investigations were performed and showed that the patient had abnormal calcifications and large, ill-defined lesions with an irregular margin on the left hip extended to the left gluteal area with skin ulceration suggestive of primary tumor calcinosis. Medical therapy has started, and a follow-up appointment was given to her in a pediatric metabolic clinic. In addition, we present a brief literature review of the effect of medical and surgical treatments on tumor calcinosis.