<p>Castleman’s disease is rare lymphoproliferative disorder mostly seen in the mediastinum. We report a case of Castleman’s disease involving a single lymph node at the left level II A in a 27-year-old female whose definitive diagnosis was made only on histological examination. Isolated Castleman’s disease of the level II A lymph node is rare and a thorough clinical and histological examination is necessary to rule out the systemic form of the disease. Clinically two types of the Castleman’s disease are seen, a unicentric variety where either a single lymph node or region is involved microscopically and multicentric variety where various lymph node regions are involved. Surgical excision is the standard of management for the unicentric Castleman’s disease and has good prognosis, whereas the multicentric type is associated with systemic symptoms and has a poor prognosis. Although, microscopically, they can be classified as–hyaline vascular, plasma cell, transitional and stromal rich type, but their clinical significance is not clear.</p>