Aim:Ecchordosis physaliphora (EP) is a benign lesion arising from notochordal remnants, and it is located in the midline along the craniospinal axis from the clivus to the sacrococcygeal region. Radiological diagnosis of EP is challenging, and its management remains a dilemma. Furthermore, a review of its management options is lacking. Our aim is to discuss the diagnostic tests and management options of retroclival EPs.Material and Methods:Four patients with a retroclival EP were assessed. Computerized tomography (CT) and magnetic resonance imaging (MRI) findings were examined thoroughly. Diffusion MRI characteristics were also studied. Furthermore, our management protocol has been discussed. Results:Four patients with a retroclival EP exhibited similar imaging findings. CT revealed bone changes and a stalk-like connection between the clivus and EP in all the patients. MRI revealed a lesion that was hyperintense on T2-weighted images and hypointense on T1-weighted images. None of the lesions exhibited contrast enhancement. All lesions were surgically resected. Histopathological examination of the lesions confirmed the diagnosis of benign notochordal remnant. Conclusion:The approach and timing of surgery should be determined according to the lesion parameters in each patient. Large heterogenous lesions that have caused significant bone changes require timely surgery. Small homogenous lesions with a stalk-like connection to clivus should be closely monitored.