The glial cells of the primary olfactory nervous system, olfactory ensheathing cells (OECs), are unusual in that they rarely form tumors. Only 11 cases, all of which were benign, have been reported to date. In fact, the existence of OEC tumors has been debated as the tumors closely resemble schwannomas (Schwann cell tumors), and there is no definite method for distinguishing the two tumor types. OEC transplantation is a promising therapeutic approach for nervous system injuries, and the fact that OECs are not prone to tumorigenesis is therefore vital. However, why OECs are so resistant to neoplastic transformation remains unknown. The primary olfactory nervous system is a highly dynamic region which continuously undergoes regeneration and neurogenesis throughout life. OECs have key roles in this process, providing structural and neurotrophic support as well as phagocytosing the axonal debris resulting from turnover of neurons. The olfactory mucosa and underlying tissue is also frequently exposed to infectious agents, and OECs have key innate immune roles preventing microbes from invading the central nervous system. It is possible that the unique biological functions of OECs, as well as the dynamic nature of the primary olfactory nervous system, relate to the low incidence of OEC tumors. Here, we summarize the known case reports of OEC tumors, discuss the difficulties of correctly diagnosing them, and examine the possible reasons for their rare incidence. Understanding why OECs rarely form tumors may open avenues for new strategies to combat tumorigenesis in other regions of the nervous system.