An 18-year-old man presenting with paraplegia due to spinal epidural infiltration at D6-D7 underwent laminectomy and spinal decompression followed by near-complete resolution of neurological symptoms; but this resolution was followed by progressively enlarging cervical lymph nodes. Leucocytosis, an elevated erythrocyte sedimentation rate, a marked polyclonal hypergammaglobulinemia and a moderate hepatosplenomegaly were found. The diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease was established histologically by lymph node biopsy about 2 years after presentation.