EBV-driven PTLD is more common after pediatric small bowel transplant (SBT) than after transplant of other organs, attributed to high graft lymphocyte density and necessarily greater immunosuppression.  Because knowledge about risks, histopathology, and outcomes of PTLD after SBT remains incomplete, we offer our experience.  Of 83 recipients transplanted between 12/2003 and 8/2012 (age-range at transplant = 2 months to 20 years; median 2 years), 9 (11%) developed PTLD. Only 2/9 with PTLD had liver-inclusive grafts, and 7/9 were female.  All 9 expressed EBV with only 1 EBV seropositive before SBT.  Median time from initial EBV DNA detection after SBT to PTLD diagnosis was 3 months.  EBV levels exceeded 200 copies/10⁵ WBC in 7/9 but were always low or undetectable before PTLD diagnosis in 2/9.  PTLD developed despite immunosuppressive taper and falling EBV levels in 6/9.  Morphology was diffuse large B-cell lymphoma in 8 and polymorphic in 1; brain masses were present in 4.  Therapy included rituximab in all and cyclophosphamide and methotrexate in most; 4 died (44%) including 3 with uncontrolled disease.  Compared to 74 patients who did not develop PTLD, those who did were older (median age 4 years vs. 1 year, p <0.01), less likely to have a liver graft (22% vs. 59%, p = 0.076), and more likely to have received rabbit-anti-thymocyte globulin (r-ATG) (67% vs. 19%, p < 0.01) and sirolimus (100% vs. 38%, p < 0.001).  Those with PTLD were not more likely to have had previous rejection (33% vs. 26%, NS).  In conclusion, PTLD after SBT appears to differ clinically and morphologically from PTLD after other transplants, with presentation at higher grade and with more frequent CNS disease.  EBV genome monitoring has limitations in predicting PTLD after SBT, and both r-ATG and sirolimus appear to increase the risk.