Hereditary spherocytosis is rarely seen to occur simultaneously in association with lymphomas or hematological malignancies. Here in, we describe a case of hereditary spherocytosis associated with diffuse follicular Non-Hodgkin lymphoma.A 54-year-old lady had presented to the emergency room of National Liver Institute Hospital, Menoufia University by marked shortness of breath, dizziness and icterus. The patient was leading a completely healthy life till three years ago when she was hospitalized for a similar attack of dyspnea and jaundice. She received 5 units or more of blood and was discharged in a fair condition without any further work up. Six months earlier; she startedtosuffer anorexia,significantweightloss alongwithsevere bony pains all over the body, but no fever, abdominal pain, lymph node swellings or bleeding. The clinical examination revealed a cachectic woman with bounding pulse, jaundice and huge splenomegaly crossing the midline. Laboratory investigations revealed marked anemia with elevated MCV and MCHC (132% and 45%) though, serum vitamin B12 and folic acid were normal (Table 1). The evident indirect hyperbilirubinemia, significant reticulocytosis, and elevated serum LDH were associated with negative direct and indirect Coomb's tests. Abdominal sonography demonstrated huge splenomegaly (29 cm) with Received: November 07, 2017; Accepted: December 01, 2017; Published: December 06, 2017 Hereditary Spherocytosis Evolving to Non-Hodgkin Lymphoma Abstract Hereditary spherocytosis is rarely seen to occur simultaneously in association with lymphomas or hematological malignancies. Here in, we describe a case of hereditary spherocytosis associated with diffuse follicular Non-Hodgkin lymphoma. Keywords: Spherocytosis; Lymphomas;Hematologicalmalignancies;Non-Hodgkin lymphoma homogenous echo-pattern along with calcular gall bladder. Furthermore, red cell distribution width (RDW) was found to be surmounted to 17% in favor of the diagnosis of HS. Osmotic fragility test was increased (showed early RBCs hemolysis at low