Idiopathic CD4 lymphocytopenia is a condition characterized by low CD4 counts. It is rare and most of the information about this illness comes from case reports. Presentation is usually in the 4th decade of life with opportunistic infections, autoimmune disease or neoplasia. The pathophysiology of this condition is not well understood. Management revolves around treatment of the presenting condition and close follow﻿-up of these patients. This review presents a narrative summary of the current literature on idiopathic CD4 lymphocytopenia.