Sacrococcygeal neoplastic lesions are very rare entities and therefore, a diagnostic and therapeutic challenge. They include developmental cysts, inflammatory, neurogenic, osseous and miscellaneous lesions. In this review, we discuss the incidence, symptoms, characteristics, diagnostic methods and treatment strategies of these enigmatic lesions. We have to highlight the high index of suspicion that is necessary when dealing with chronic sacrococcygeal pain or multiple perianal abscesses or persistent perianal drainage, because any misinterpretation may result in unfavorable outcomes.