Acute lymphoblastic leukemia is a hematological malignancy of lymphoid progenitor cells associated with excessive proliferation of lymphocytes, and lymphocyte markers can be used for the diagnosis and assessment of disease. Cell surface receptors, including T cell receptor and immunoglobulin (on T and B cells, respectively), are among the most important lymphocyte markers. Gene segment variation, i.e., involvement of several genes in the expression of each receptor, is effective on extensive rearrangement of cell surface receptors. These receptors can be observed in both T-acute lymphoblastic leukemia (ALL) and precursor B-ALL cells. In this study, we have discussed T cell receptor (TCR) gene rearrangements in ALL. As leukemia cell proliferation originates from a unique clone, clone-specific rearrangement can be very helpful in diagnosis and detection of remaining malignant cells from among normal cells for minimal residual disease. On the other hand, each of the TCR genes is associated with translocation in ALL types, and the result of gene rearrangements can be used as prognosis markers.