Melanoma diagnosed during pregnancy is a rare clinical case presentation which must be mastered. In the absence of guidelines for this clinical challenge, we performed a review of the literature and provide a practical guideline on how to manage such rare clinical cases based on our clinical experience. Expecting mothers require adequate counselling and explanation of all therapeutic options as they take responsibility for more than their own lives. However, they should be guided through the process of diagnostic and therapeutic measures in a potentially life-threatening situation. Pregnancy itself is no reason to withhold any type of necessary melanoma surgery. Perioperative management, however, requires certain adjustments in order to comply with this special situation. If indicated, even adjuvant and palliative systemic therapy need to be given to the patient, but they also have to be adapted to the specific circumstances as data is still sparse, especially for the new first and second line therapies with antibodies and targeted molecules.