Castleman's disease (CD) is a enigmatic lymphoid disease of unknown etiology which rarely manifest itself as an isolated pelvic mass. We report a case of pelvic Castleman's disease masquerading as a uterine myoma. The patient presented symptoms related to compression of adjacent structures, splenomegaly and abdominal lymphadenopathy, the laboratory data revealed positive Epstein-Barr virus serology, elevated beta 2-microglobulin level and presence of antinuclear antibodies. The intraabdominal involvement, histological patterns and clinical forms of this condition are reviewed. Likewise etiopathogenic, radiologic and therapeutic aspects related with this entity are discussed. We suggest that pelvic Castleman's disease should be included in the differential diagnosis of females presenting a pelvic mass containing calcifications.