Splenic marginal zone lymphoma (SMZL) is a low-grade mature B-cell lymphoma that typically presents in the form of splenomegaly and lymphocytosis. The diagnosis is traditionally made through splenic histology, the presence of circulating villous lymphocytes, or bone marrow biopsy. Its treatment can be in the form of chemotherapy, such as rituximab, or active surveillance. This case presentation discusses a 76-year-old female with a long history of hydroxyurea use for an unknown reason presenting with atypical symptoms requiring bone marrow biopsy to diagnose SMZL. This unique case demonstrates the importance of further research and studies into atypical SMZL presentations and hydroxyurea's potential in precipitating secondary malignancies.