Abstract Most of neuroendocrine neoplasms (NENs) are located in the gastrointestinal tract and lung, and they are rarely found on the upper aero‐digestive tract, which limit the current literature about nasopharyngeal NENs. This systemic review will summarize the clinical, pathological features and optimal diagnosis and management of different types of nasopharyngeal NENs (NP NENs). In‐addition, we herein report an EBV negative TP53‐mutated/ Rb‐wild type nasopharyngeal neuroendocrine carcinoma (NEC) in a young man in which touch preparation cytology studies were integral to establishing a definitive diagnosis. To our knowledge, only very few cases of primary neuroendocrine carcinoma of the nasopharynx have been reported in the literature and the reports of these cases have not included detailed description of different types and how to optimally diagnose and manage them. In this abstract, we also highlighted the evidence about the safety of using growth factors in patients with sickle cell anemia who are receiving cytotoxic chemotherapy.