A case of periodic fever and persistent splenomegaly in a 2-year-old boy

We report an unusual case of periodic fever associated with persistent splenomegaly in a 2-year-old boy. Febrile episodes occurred at regular 2 weekly intervals and each lasted for 1–2 days. Following resolution of the fever on each occasion, the patient developed neck stiffness of 1–2 days duration. The periodic febrile episodes, as well as the splenomegaly, spontaneously resolved 9 months after the onset of disease. Infectious and malignant causes were ruled out as far as possible. This patient’s clinical features are unusual and do not match any known category of periodic fevers in childhood. We believe this case highlights the diagnostic challenges periodic fevers commonly represent.