Tumoral calcinosis is a rare clinical and histopathologic syndrome that causes the formation of calcium salt crystal deposits in periarticular soft tissue. This soft tissue calcification typically behaves as a large-sized tumor. However, the mechanism is unknown for the massive periarticular calcification caused by tumoral calcinosis. In addition, tumor calcinosis occurs infrequently in patients who have been on long-term hemodialysis and have end-stage renal disease (ESRD). This report describes a 40-year-old male uremia patient on long-term hemodialysis. This patient developed a huge tumor mass and pain over the left shoulder and was diagnosed with tumoral calcinosis. Although common treatments include management of metabolic levels such as calcium and phosphate, in some cases, surgical intervention may be needed. In this patient case, complete surgical excision was done successfully with an excellent outcome at the 2-year follow-up.