During the last 12 years, tumoral calcinosis (TC) has become an entity that is better understood by radiologists, clinicians, and pathologists. Several aspects of TC are well documented by the scientific community, although the most important, etiology, remains an enigma. As a consequence, the treatment of TC continues to be symptomatic. Imaging continues to be the best method for diagnosis, assessing extension, evaluating complications, monitoring treatment, and selecting patients for surgical excision. Investigators from different countries are reporting the most elusive component of TC: calcific myelitis. New systemic and cerebrovascular complications, even in the young, have been described recently. Similarly, ocular manifestation, which had not been reported previously, is well described in the recent literature. A few, poorly documented reports are confounding TC myelitis with osteomyelitis and TC periarticular soft tissue masses with tumoral calcium pyrophosphate dihydrate (CPPD) of the temporomandibular joint and spine. Advances in the clinical arena have resulted in a new approach and classification of several clinical forms of TC. Most of the recent literature has been related to the histopathology of TC. A new view of the old "traumatic theory" suggests microtrauma of connective tissues around the joints, causing bleeding and the formation of rudimentary bursae as the initial event. Perhaps, in the near future, research will define at the genetic and molecular levels the etiology and pathophysiology that will allow the treatment and prevention of TC.