Lymphangiomatosis of the spleen with diffuse lymphangiomatosis of the bone is extremely rare and there is no consensus about management at present. Here, the authors report a child presenting initially with back pain and diagnosed as lymphangiomatosis on several sites throughout the bony structures and the spleen. The patient is in a stable state at the end of 8 years without any treatment. The authors also discuss the follow-up option that may be considered as a strategy for management.