As individuals with germ line predisposition to hematologic malignancies are diagnosed with increasing frequency, the need for clinical surveillance has become apparent. Unfortunately, few prospective data are available, so recommendations are based on collective experience and expert consensus. There is general agreement to advocate for expert consultation or referral of patients to centers with expertise in these syndromes, since presentations and disease progression can be subtle, and treatment strategies must be tailored. Here, we summarize and integrate expert consensus recommendations and medical management considerations for the patient newly diagnosed with a leukemia predisposition disorder. Indications to consider additional studies and referral for allogeneic stem cell transplantation are also discussed.