OBJECTIVE: To analyze the clinical characteristics and MRI imaging of the patients with pituitary stalk interruption syndrome (PSIS) in the First Affiliated Hospital of Anhui Medical University in the past four years, and to achieve better comprehension of this disease. METHODS: Ten patients with PSIS (9 males, 1 female) in our hospital were retrospectively analyzed, regarding clinical manifestation, laboratory data and MRI imaging. RESULTS: The clinical features of 10 cases of PSIS were as follows: growth retardation, 55% of patients with hypogonadism, 45% of patients with short stature; the dystocia rate at birth is as high as 90%, of which 61% are breech presentation and 27% are foot presentation; 10 patients with PSIS, the height was between 135 cm and 180 cm, the weight was between 31 kg and 93 kg, the lower part was larger than the upper part, and the finger distance was smaller than the height; bone age is behind 3~7 years old; normal intelligence; 10 patients have clinical manifestations of hypopituitary hypofunction; no manifestations of diabetes insipidus; MRI imaging of pituitary showed that the pituitary stalks were not shown, atrophy or aplasia of anterior pituitary, posterior pituitary ectopic. CONCLUSION: The incidence of PSIS is low, and the etiology and pathogenesis are unknown. Appropriate hormonal replacement therapy is the only effective way but the timing of treatment is very important. Therefore, clinical doctors should strengthen the awareness of the disease, and master the clinical characteristics of the disease to grasp the timing of treatment.