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Rosai-Dorfman disease (RDD) is a rare, benign non-Langerhans cell histiocytosis predominantly affects lymph nodes and skin. Despite its benign nature, RDD can cause serious hematological complications. A 14-year-old male, presented with 3-month history of hemolytic anemia, lymphadenopathy, hepatosplenomegaly and rash. After thorough investigation, RDD was diagnosed by mediastinal lymph node biopsy that revealed presence of S100 and CD68-positive cells with absence of CD1a confirming the diagnosis of RDD. Treatment involved combination of steroids and Rituximab, which proved to be highly effective. The patient had dramatic improvement and entered remission, with follow-up period of 2 years. It is important to note that although RDD is a rare disease, it causes severe complications, as evidenced by the patient's parameters. Thus, prompt diagnosis and treatment are paramount. Histological diagnosis is of great value, as it helps confirming and guiding treatment decisions. With the right treatment, patients can experience great recovery and quality of life., Competing Interests: There is no conflict of interest., (© The Author(s) 2023. Published by Oxford University Press.)