Abstract. Palmar fasciitis and polyarthritis (PFPA) is an uncommon disorder clinically characterized by rapidly developing bilateral arthritis of the hands and fasciitis of the palms. This cancer-associated syndrome, primarily linked to ovarian cancer, has also been associated with multiple different malignancies. PFPA symptoms usually precede the detection of ovarian cancer. In all patients from case series and single case reports described, ovarian cancer is already present at an advanced stage. Therefore, the authors aimed to present a case and systematically review available evidence on the association between PFPA and ovarian cancer, as gynecological oncologists. Awareness and recognition of PFPA may allow for earlier diagnosis and treatment of an occult ovarian cancer. The commitment of the attending gynecologists to become familiar with and identify rare diseases may lead to the detection at an early and curative stage and potentially life-saving therapeutic interventions.