Castleman disease (CD; synonym: angiofollicular lymph node hyperplasia) is a rare disorder of the lymphoid system of uncertain etiology. The disease shows two clinical (unicentric and multicentric) and three histomorphological (hyaline vascular, plasma cell-rich and mixed) forms which may present in varying combinations. The mediastinum and the abdominal cavity are the main sites of predilection for CD but any other nodal or extra-nodal site can be affected. We herein describe four cases of unicentric hyaline-vascular CD located in the abdomen and treated by surgical excision. Patients were two women and two men with a mean age of 42 years (range, 19-55). None had evidence of Human immunodeficiency virus (HIV) infection. Symptoms were nonspecific and diagnosis was not achieved prior to surgery in any of the patients. The lesions measured 4-10 cm (mean, 6 cm). Histologically, all were consistent with the hyaline vascular type of CD. On last follow-up (mean, 90 months; range, 24-134), one patient developed intra-abdominal desmoid tumor of the mesentery (3 years after surgery for CD) but none had evidence of recurrence and none developed multicentric manifestation of CD at other sites. In conclusion, surgical removal of unicentric CD in non-HIV patients is curative and help to verify diagnosis. Given that local recurrence of unicentric CD is uncommon, even after incomplete surgical resection, newly appearing lesions detected on follow-up should be biopsied to rule out other metachronous neoplasms as seen in one of our patients.