Myelolipomas are benign tumors, consisting of hematopoietic cells and mature adipose tissue, which mainly occur within the adrenal gland. Extra-adrenal myelolipomas are rare, and fewer than 60 cases have been reported in the literature. Here, we report a case of intrasplenic myelolipoma in a 42-year-old man with more than 1 month of abdominal pain. Computed tomography scanning revealed a giant, heterogeneous, well-demarcated mass in the spleen. Splenectomy was performed, and an intrasplenic giant mass was completely excised. The diagnosis of myelolipoma was made based on morphological examination. To the best of our knowledge, this is the third reported case of myelolipoma in the human spleen.