Blastic plasmacytoid dendritic cell neoplasm is a rare hematopoietic malignancy with a poor prognosis that is seen primarily in the elderly population. We describe a pediatric patient with blastic plasmacytoid dendritic cell neoplasm who subsequently developed Guillain Barre syndrome followed by hemophagocytic lymphohistiocytosis. All 3 conditions are uncommon, particularly in the pediatric population. It is unclear whether this patient developed these disease states independently, whether they were due to a viral trigger or if she has an underlying immune dysfunction that could have contributed to the development of these conditions. The patient is currently in remission and awaiting further immune work-up.