Cryptococcal meningitis (CM) is a rare condition in patients with primary nephrotic syndrome (PNS). Here, we describe the clinical characteristics, possible risk factors, and outcomes of eleven patients with PNS and CM. A systematic review of medical records from 11 patients with PNS and CM admitted to the First Affiliated Hospital of Wenzhou Medical University from April 2005 to April 2015 was performed. A total of 30 cases were randomly selected as controls from PNS inpatients without infection during the same period. The mean age at presentation was 64.6 years (range: 47.8-72.2 years), and the female-to-male ratio was 5:6. The mean duration of PNS was 14.0 months (range: 1-25 months); the cumulative prednisone dose over the preceding year was 3.8±1.98g; the mean albumin level was 25.8±10.1g/L; the mean serum creatinine level was 116.4±64.3µmol/L. Three patients died, 1 patient survived with sequelae, and 7 patients survived without sequelae. Unfavorable outcomes were associated with low serum albumin levels (p=0.004) and higher cumulative prednisone dose over the preceding year (p=0.025). Compared with PNS patients without infections, those with CM were older (p < 0.001) and usually had higher serum creatinine levels (p < 0.001). Old age and high serum creatinine levels are important risk factors for the development of CM in patients with PNS. .