Background Primary laryngeal cryptococcosis (PLC) is a rare disease, with only 41 reported cases since 1975. Though cryptococcaemia affects immunosuppressed patients, PLC tends to occur in immunocompetent people, with inhaled corticosteroid (ICS) use a major risk factor. Cases typically present with hoarseness and may be mistaken for malignancy. Diagnosis relies on specific stains for Cryptococcus spp. on histopathology. Methods We present 7 cases of PLC, from the records of four Sydney ENT surgeons, which is the largest series of PLC cases ever. We also review the 41 previously reported cases. Results The average age was 65 (range: 30 – 88) with a slight female preponderance (57%). Most cases were from USA (37%) or Australia (32%). The main risk factor was ICS use (59%). 18% of patients were immunosuppressed (HIV/transplant), 15% used oral steroids and 15% were diabetic. 33% of patients smoked. 21% reported exposure to bird faeces, Eucalyptus or gardening. All patients presented with hoarseness, which was the only presenting complaint for 38%. A dry cough was the next most common feature (28%). No patients had signs of pulmonary or meningeal involvement. Suspicion for malignancy was explicitly cited as a concern in 15% of cases, though all cases were biopsied with diagnosis usually confirmed with histopathological stains. Serum Cryptococcal antigen was 23% sensitive. PLC prevalence appears to be increasing, perhaps due to increased ICS use, with more cases reported in the past 8 years alone than in the preceding 22 years. Oral fluconazole remains the mainstay of treatment with 92% of cases responding completely or almost completely. Conclusion PLC is a rare cause of persistent hoarseness, which should be considered in patients with a history of ICS use or immunosuppression. Diagnosis must be made on biopsy. Patients typically respond well to prolonged fluconazole treatment with laser ablation reserved for recurrent or refractory lesions.