Objective Posttransplant lymphoproliferative disorder (PTLD) is a unifying term for a spectrum of lymphoid expansion entities brought about by immunosuppression and is strongly associated with Epstein-Barr virus (EBV). Otolaryngological findings tend to present early in the clinical course; therefore, disease awareness among otolaryngologists is of utmost importance. This review synthesizes the body of literature pertaining to PTLD involving the head and neck, summarizes contemporary management, and highlights areas for future research. Data Sources PubMed/Medline. Review Methods Primary literature search of the Medline database was performed for all titles published in the past 10 years pertaining to PTLD. The database search included PTLD combined with a collection of otolaryngological MeSH terms. Full manuscripts were reviewed based on relevance of their title and abstract. Selection into this review was according to clinical and scientific relevance. Conclusion Adenotonsillar focus is common in children in whom adenotonsillectomy may be diagnostic and prevents potentially morbid airway obstruction. Sinonasal PTLD may mimic fungal infection. Laryngotracheal involvement predominately presents in children with symptoms of airway obstruction. PTLD limited to the esophagus is rare. Oral PTLD is rare and phenotypically varied. Cutaneous presentation of PTLD is infrequent, yet one-third of cases affects the head and neck. PTLD may present as cervical lymphadenopathy. Implications for Practice PTLD consideration is vital when evaluating posttransplant patients. Children and EBV-seronegative patients should receive otolaryngological follow-up after transplant. PTLD treatment is multidisciplinary and typically led by lymphoma specialists. Formal partnerships between otolaryngologists and transplant centers may improve patient care and research quality.