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Objectives To report a study of a complex case of a relapsing non-Hodgkin lymphoma (NHL) overlapping with a polysymptomatic Undifferentiated Connective Tissue Disease (UCTD). The former is a hematological malignancy, and the latter and autoimmune disease (AD). Both are believed to share a genetic background, as both are etiologically linked to genetic variations of the major histocompatibility complex (MHC). Indeed, individuals with ADs harbor a higher risk of developing NHL.Methods Medical history of the patient had begun at the age of 48 years old, when developed acute bulbar symptoms (bilateral facial palsy, dysphagia), fever and long tract symptoms (leg weakness). The condition was diagnosed as polyradiculoneuritis and treated by plasmapheresis. After being discharged, he had regained some weight, and motor strength, yet developed panuveitis, which raised a suspicion of Behçet’s disease. Workup showed that opthalmological presentation was only part of a multisystem disease and systemic Systemic lupus erythematosus was hypothesized. Eventully UCTD with antiphospholipid syndrome (APS) was diagnosed and the patient started on corticosteroids, to which he had a favorable clinical response. Over the next two years he developed lymphadenopathy and hepatosplenomegaly, and eventually a follicular T-cell lymphoma was diagnosed, at 50 years old, and treated by a NHL R-CHOP regimen. Also, he had psoriatic relapses and kept losing weight. Over the next two years he was diagnosed with achalasia and subsequently treated successfully by esophageal dilatation. The latest reason for referral to the immunological outpatient clinic, at 54 years old, was right hip pain.Results Clinical presentation was unremarkable, apart from palmar psoriasis, and right hip pain. The pain was not triggered by physical activity, was mostly nocturnal, and made the patient sleep on the left hip. He had a therapy of 5 mg prednisone, vitamins B12 and D, a proton-pump inhibitor, and topical eye drops. Malignancy relapse was suspected and confirmed after a femur biopsy.Conclusions The case adds to the body of evidence on the association between NHL and UCTD and emphasizes the importance of keeping both in mind when dealing with a chronic course of either one of the conditions.