Staghorn stones have always been a challenge for urologists, especially in some special situations, such as horseshoe kidney, ectopic kidney, paediatric kidney, and solitary kidney. The treatment of these staghorn stones must be aggressive because they can lead to renal function loss and serious complications. The gold-standard management for staghorn stones is surgical treatment with the aim of clearing the stones and preserving renal function. Treatment methods for staghorn stones have developed rapidly, such as extracorporeal shock wave lithotripsy, retrograde intrarenal surgery, percutaneous nephrolithotomy and laparoscopy and open surgery. Whether the standard procedures for staghorn stones can also apply to these stones in special situations is still not agreed upon. The decision should be made individually according to the circumstances of the patient. In this review, we evaluates the previous studies and comments on the management of staghorn stones under special situations in the hope of guiding the optimal choice for urologists.