We report the unusual case of a 43-year-old man with a diagnosis of cl
inical stage I A mixed cellularity Hodgkin's disease (HD), who relapse
d 4 years after diagnosis with exclusive bone marrow involvement and a
cyclic variation in body temperature typical of Pel-Ebstein fever. In
the absence of clinical and laboratory signs of infection, a restagin
g of the lymphoma was performed. Total-body CT scan revealed no parenc
hymal or lymph node involvement, while a bone-marrow biopsy was positi
ve for the presence of Reed-Sternberg cells. Therefore, the patient wa
s started on combination chemotherapy, which promptly induced a normal
ization of the temperature curve. The presence of typical Pel-Ebstein
fever, which is reported to be very rare, in association with bone mar
row localization as the only site of relapse, suggests a relationship
between these two rare manifestations of the disease.